<clinical_study>
  <!-- This xml conforms to an XML Schema at:
    https://clinicaltrials.gov/ct2/html/images/info/public.xsd -->
  <required_header>
    <download_date>ClinicalTrials.gov processed this data on September 20, 2023</download_date>
    <link_text>Link to the current ClinicalTrials.gov record.</link_text>
    <url>https://clinicaltrials.gov/ct2/show/NCT04447846</url>
  </required_header>
  <id_info>
    <org_study_id>IRB00204427</org_study_id>
    <nct_id>NCT04447846</nct_id>
  </id_info>
  <brief_title>Novel Cognitive Treatment Targets for Epidiolex in Sturge- Weber Syndrome</brief_title>
  <official_title>Novel Cognitive Treatment Targets for Epidiolex in Sturge- Weber Syndrome: A Phase II Trial</official_title>
  <sponsors>
    <lead_sponsor>
      <agency>Anne Comi, MD</agency>
      <agency_class>Other</agency_class>
    </lead_sponsor>
    <collaborator>
      <agency>Jazz Pharmaceuticals</agency>
      <agency_class>Industry</agency_class>
    </collaborator>
    <collaborator>
      <agency>Faneca 66 Foundation</agency>
      <agency_class>Other</agency_class>
    </collaborator>
  </sponsors>
  <source>Hugo W. Moser Research Institute at Kennedy Krieger, Inc.</source>
  <oversight_info>
    <has_dmc>Yes</has_dmc>
    <is_fda_regulated_drug>Yes</is_fda_regulated_drug>
    <is_fda_regulated_device>No</is_fda_regulated_device>
    <is_us_export>Yes</is_us_export>
  </oversight_info>
  <brief_summary>
    <textblock>
      The purpose of this study is to better understand the utility of cannabidiol (CBD/ Epidiolex)&#xD;
      for improving the treatment of cognitive impairments in Sturge-Weber syndrome (SWS).&#xD;
    </textblock>
  </brief_summary>
  <detailed_description>
    <textblock>
      The investigators hope to gain an understanding of the utility of pharmaceutical grade CBD&#xD;
      used for the treatment of cognitive impairments in SWS in this open-label study. Anecdotal&#xD;
      evidence from a phase I trial investigating the use of CBD for medically refractory seizures&#xD;
      suggests CBD may also have a beneficial effect on cognition, mood, and behavior. The&#xD;
      investigators hypothesize that CBD/ Epidiolex will improve SWS brain function resulting in&#xD;
      improved cognitive function, social interactions, mood, motor function and behavior, as well&#xD;
      as reduced migraines. This is an open-label prospective oral drug trial of Epidiolex in 10&#xD;
      subjects. Assessments will be done at baseline and repeated after 6 months on study drug.&#xD;
    </textblock>
  </detailed_description>
  <overall_status>Completed</overall_status>
  <start_date type="Actual">October 14, 2019</start_date>
  <completion_date type="Actual">December 9, 2022</completion_date>
  <primary_completion_date type="Actual">August 16, 2021</primary_completion_date>
  <phase>Phase 2</phase>
  <study_type>Interventional</study_type>
  <has_expanded_access>No</has_expanded_access>
  <study_design_info>
    <allocation>N/A</allocation>
    <intervention_model>Single Group Assignment</intervention_model>
    <primary_purpose>Treatment</primary_purpose>
    <masking>None (Open Label)</masking>
  </study_design_info>
  <primary_outcome>
    <measure>List Sorting Working Memory Test</measure>
    <time_frame>Baseline, Follow-up (6 months)</time_frame>
    <description>Data on cognitive function was collected using the List Sorting Working Memory Test from the NIH Toolbox. Data was collected on working memory performance, which was transformed into a t-score from 0 to 100 where a higher t-score indicates better performance. T-score of 50 indicates the population mean with a standard deviation of 10. Data was collected at baseline and after 6 months on study drug.</description>
  </primary_outcome>
  <secondary_outcome>
    <measure>Picture Vocabulary Test</measure>
    <time_frame>Baseline, Follow-up (6 months)</time_frame>
    <description>Data on cognitive function was collected using the Picture Vocabulary subtest from the NIH Toolbox. Single words are presented via an audio file, paired simultaneously with 4 screen images of objects, actions, and/or depictions of concepts. The task is to pick the picture that matches the spoken word. Performance on the task was transformed into a t-score from 0 to 100 where a higher t-score indicates better performance. T-score of 50 indicates the population mean with a standard deviation of 10. Data was collected at baseline and after 6 months on study drug.</description>
  </secondary_outcome>
  <secondary_outcome>
    <measure>Seizure Frequency</measure>
    <time_frame>Baseline, Follow-up (6 months)</time_frame>
    <description>Change in seizure frequency by seizure score at pre-treatment baseline and after six months. The seizure score is taken from the Sturge-Weber Neuroscore on a scale of 0 to 4 where 0=none, 1=1+. but controlled, 2=Breakthrough, 3=monthly, 4=Weekly+. Higher scores indicate worse outcome.</description>
  </secondary_outcome>
  <secondary_outcome>
    <measure>Migraine Severity</measure>
    <time_frame>Baseline, Follow-up (6 months)</time_frame>
    <description>Data on migraine severity will be collected using patient responses to questions on a standard six-point scale. Data will be collected on the frequency of an event (e.g. feelings of frustration, performance of daily activities) which will be transformed into a score from 0 to 100 where higher scores indicate a less migraine severity and better outcomes. Data will be collected at baseline and after 6 months on study drug.</description>
  </secondary_outcome>
  <secondary_outcome>
    <measure>Modified House Classification Scores</measure>
    <time_frame>Baseline, Follow-up (6 months)</time_frame>
    <description>Data on motor function was collected using a Modified House Classification. Data was collected on the ability to complete a task with the subject's non-dominant hand which was transformed into a score from 1 to 8 and 0 to 32 where higher scores indicate better motor function. Data was collected at baseline and after 6 months on study drug.</description>
  </secondary_outcome>
  <secondary_outcome>
    <measure>Erhardt Developmental Prehension Assessment Scores</measure>
    <time_frame>Baseline, Follow-up (6 months)</time_frame>
    <description>Data on motor function was collected using the Erhardt Developmental Prehension assessment. Data was collected on the ability to complete a task with each hand, which was scored as age equivalence of task performance (in months). Higher scores indicate better motor function. Data was collected at baseline and after 6 months on study drug.</description>
  </secondary_outcome>
  <secondary_outcome>
    <measure>Pediatric Evaluation of Disability Inventory Computer Adapted Test</measure>
    <time_frame>Baseline, Follow-up (6 months)</time_frame>
    <description>Data on motor function was collected using the Pediatric Evaluation of Disability Inventory Computer Adapted Test. Data was collected on the subject's ability to complete tasks involved in daily activities, mobility, and social/ cognitive activities. The data was transformed into scaled scores ranging from 20 to 80, where higher scores indicate better motor function. Data was collected at baseline and after 6 months on study drug.</description>
  </secondary_outcome>
  <secondary_outcome>
    <measure>ABILHAND Questionnaire</measure>
    <time_frame>Baseline, Follow-up (6 months)</time_frame>
    <description>Data on motor function was collected using the ABILHAND questionnaire, a measure of manual ability for adults with upper limb impairments. Data was collected on the subject's ability to complete daily activities that involve the upper limbs. Score was collected as a patient measure with scores ranging from -10 to 10. Higher scores indicate better motor function. Data was collected at baseline and after 6 months on study drug.</description>
  </secondary_outcome>
  <secondary_outcome>
    <measure>Wechsler Intelligence Scale for Children, Fifth Edition (WISC-V) or Wechsler Adult Intelligence Scale (WAIS-IV)</measure>
    <time_frame>Baseline, Follow-up (6 months)</time_frame>
    <description>Data on cognitive function was collected using selected subtests, from either the Wechsler Intelligence Scale for Children, Fifth Edition (WISC-V) or the Wechsler Adult Intelligence Scale (WAIS-IV). For the WISC-V and WAIS-IV subtests selected, scaled scores were derived from the normative data which account for age and demographic information. The selected WISC-V/WAIS-IV subtests were as follows: Digit Span, Symbol Search, Coding and Processing Speed; the first three subtests were scored from 0-10, the fourth subtest from 0-100, and for all subtests higher score is better. The selected subtests, from the WISC-V and the WAIS-IV, were combined to increase statistical power.&#xD;
Statistically rare changes in individual test scores were determined using a reliable change index methodology based upon normative information for these assessments.</description>
  </secondary_outcome>
  <secondary_outcome>
    <measure>Pediatric Neurological Quality of Life (Neuro-QoL)</measure>
    <time_frame>Baseline, Follow-up (6 months)</time_frame>
    <description>Data on cognitive function was collected using Neurological Quality of Life scales from the NIH Toolbox. Data on frequency of an event (e.g. forgetting schoolwork) was collected and transformed into a t-score from 0 to 100 where higher t-scores indicate worse anger, worse anxiety, worse pain, better social relationships, worse stigma, worse depression, better cognitive function, and worse fatigue. T-score of 50 indicates the population mean with a standard deviation of 10. Data was collected at baseline and 6 months on study drug.</description>
  </secondary_outcome>
  <secondary_outcome>
    <measure>Behavior Rating Inventory of Executive Function, Second Edition (BRIEF-2)</measure>
    <time_frame>Baseline, Follow-up (6 months)</time_frame>
    <description>Data on executive function was collected using the Behavior Rating Inventory of Executive Function, Second Edition (BRIEF-2). Data on the frequency of an event (e.g. becomes upset too easily) was collected and transformed into a t-score. The following BRIEF-2 subtests were evaluated: Behavioral Regulation Index, Emotional Regulation Index, Cognitive Regulation Index, and Global Executive Composite. For each BRIEF-2 subtest, t-scores range from 0 to 100; a score of 50 indicates the population mean with a standard deviation of 10. For all BASC-3 subtests, higher scores are worse outcome.</description>
  </secondary_outcome>
  <secondary_outcome>
    <measure>Social Responsiveness Scale, Second Edition (SRS-2)</measure>
    <time_frame>Baseline, Follow-up (6 months)</time_frame>
    <description>Data on social function was collected using the Social Responsiveness Scale-Second Edition (SRS-2). Data on a child's ability to engage in emotionally appropriate reciprocal social interactions in naturalistic settings was collected and transformed into a t-score from 0 to 100 where higher scores indicate greater impairment in social function. T-score of 50 indicates the population mean with a standard deviation of 10. Data was collected at baseline and 6 months on study drug.</description>
  </secondary_outcome>
  <secondary_outcome>
    <measure>Behavioral Assessment System for Children, Third Edition (BASC-3)</measure>
    <time_frame>Baseline, Follow-up (6 months)</time_frame>
    <description>Data on behavioral function was collected using the Behavioral Assessment System for Children, Third Edition (BASC-3). Data on the frequency of a behavior (e.g. avoids eye contact) was collected and transformed into a t-score. Subscales for the BASC-3 scored were: External Problems Composite, the Internal Problems Composite, the Behavioral Symptoms Index, and the Adaptive Skills Composite. For the first three subscales, lower t-score indicates better outcome; for the fourth, a lower t-score indicates worse outcome. T-scores for all the BASC-3 subscales range from 0 to 100, and a t-score of 50 indicates the population mean with a standard deviation of 10.</description>
  </secondary_outcome>
  <secondary_outcome>
    <measure>Screen for Child Anxiety Related Disorders (SCARED)</measure>
    <time_frame>Baseline, Follow-up (6 months)</time_frame>
    <description>Data on anxiety was collected using the Screen for Child Anxiety Related Disorders (SCARED). Data on the truthfulness of a statement (e.g. I am nervous) was collected and transformed into a score from 0 to 82 where higher scores indicate greater anxiety. Data was collected at baseline and 6 months on study drug.</description>
  </secondary_outcome>
  <secondary_outcome>
    <measure>Quality of Life in Childhood Epilepsy Questionnaire (QOLCE-55)</measure>
    <time_frame>Baseline, Follow-up (6 months)</time_frame>
    <description>Data on quality of life was collected using the Quality of Life in Childhood Epilepsy Questionnaire (QOLCE-55). Data on the frequency of an event (e.g. had trouble concentrating on a task) was collected and transformed into a score from 0 to 100 where higher scores reflect better quality of life. Data was collected at baseline and 6 months on study drug.</description>
  </secondary_outcome>
  <secondary_outcome>
    <measure>Safety of Epidiolex</measure>
    <time_frame>Baseline, Follow-up (6 months)</time_frame>
    <description>Safety of Epidiolex was measured by the number of adverse events and serious adverse events that result from study drug.</description>
  </secondary_outcome>
  <secondary_outcome>
    <measure>Neuroscore</measure>
    <time_frame>Baseline, Follow-up (6 months)</time_frame>
    <description>Data on neurological function was collected using the Neuroscore. Data on frequency of seizures, extent of hemiparesis, assessment of visual field cut, and degree of cognitive functioning was transformed into a score from 0 to 15 where higher scores indicate worse neurologic function. Data was collected at baseline and 6 months on study drug.</description>
  </secondary_outcome>
  <secondary_outcome>
    <measure>Port-wine Birthmark Score</measure>
    <time_frame>Baseline, Follow-up (6 months)</time_frame>
    <description>Data on facial port-wine birthmarks was collected using the Port-wine Birthmark Score. Data on percent of face covered, thickness of birthmark, and darkness of birthmark color was collected and transformed into a score from 0 to 43 where higher scores indicate greater severity and greater surface area involved. Data was collected at baseline and 6 months on study drug.</description>
  </secondary_outcome>
  <secondary_outcome>
    <measure>Adult Neurological Quality of Life (Neuro-QoL)</measure>
    <time_frame>Baseline, Follow-up (6 months)</time_frame>
    <description>Data on cognitive function was collected using Neurological Quality of Life scales from the NIH Toolbox. Data on frequency of an event was collected and transformed into a t-score from 0 to 100. Higher t-scores indicate better communication, better ability to participate in social activity, worse anxiety, worse depression, worse emotional and behavioral dyscontrol, worse fatigue, better positive affect, worse sleep disturbance, better lower and upper extremity functions, worse stigma, better satisfaction with social roles, and better cognitive function. T-score of 50 indicates the population mean with a standard deviation of 10. Data was collected at baseline and 6 months on study drug.</description>
  </secondary_outcome>
  <number_of_arms>1</number_of_arms>
  <enrollment type="Actual">11</enrollment>
  <condition>Sturge-Weber Syndrome</condition>
  <arm_group>
    <arm_group_label>Cannabidiol/ Epidiolex</arm_group_label>
    <arm_group_type>Experimental</arm_group_type>
    <description>All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome.</description>
  </arm_group>
  <intervention>
    <intervention_type>Drug</intervention_type>
    <intervention_name>Cannabidiol</intervention_name>
    <description>Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.</description>
    <arm_group_label>Cannabidiol/ Epidiolex</arm_group_label>
    <other_name>Epidiolex</other_name>
    <other_name>CBD</other_name>
  </intervention>
  <eligibility>
    <criteria>
      <textblock>
        Inclusion Criteria: Participants with Sturge-Weber syndrome brain involvement as defined on&#xD;
        neuroimaging (n=10 subjects, male and female, ages 3 to 50 years of age) and the following:&#xD;
&#xD;
          -  Cognitive impairment defined as a cognitive neuroscore greater than or equal to 2 at&#xD;
             screening.&#xD;
&#xD;
          -  Anti-epileptic, mood or behavioral drugs (if on) at stable doses for a minimum of 4&#xD;
             weeks prior to enrollment.&#xD;
&#xD;
          -  If present, VNS must be on stable setting for a minimum of 3 months prior to&#xD;
             enrollment.&#xD;
&#xD;
          -  If on ketogenic or Atkins diet, must be on stable ratio for a minimum of 3 months&#xD;
             prior to enrollment.&#xD;
&#xD;
          -  Previous subjects who fail at any point to meet continuation criteria and withdraw&#xD;
             early may be considered for re-enrollment under new subject ID as long as the above&#xD;
             inclusion criteria are met. The determination of whether to re-enroll will be made by&#xD;
             the PI and sponsor on a case-by-case basis. Re-enrollment can occur no earlier than 4&#xD;
             weeks after the final, post-weaning follow-up visit under the old subject ID.&#xD;
&#xD;
          -  Written informed consent obtained from the patient or the patient's legal&#xD;
             representative must be obtained prior to beginning treatment.&#xD;
&#xD;
        Exclusion Criteria:&#xD;
&#xD;
          -  Patients with any severe and/or uncontrolled medical conditions at randomization such&#xD;
             as:&#xD;
&#xD;
               1. Liver disease such as cirrhosis, decompensated liver disease, and chronic&#xD;
                  hepatitis (i.e. quantifiable HBV-DNA and/or positive HbsAg, quantifiable HCV-RNA)&#xD;
&#xD;
               2. Uncontrolled diabetes as defined by fasting serum glucose greater than 1.5&#xD;
&#xD;
               3. Active (acute or chronic) or uncontrolled severe infections&#xD;
&#xD;
               4. Active, bleeding diathesis&#xD;
&#xD;
          -  Patients who have a major surgery or significant traumatic injury within 4 weeks of&#xD;
             study entry, patients who have not recovered from the side effects of any major&#xD;
             surgery (defined as requiring general anesthesia), or patients that may require major&#xD;
             surgery during the course of the study.&#xD;
&#xD;
          -  Patients who start or discontinue a seizure, mood or behavioral medication in the 4&#xD;
             weeks leading up to screening.&#xD;
&#xD;
          -  Prior treatment with any investigational drug or use of any other cannabis product&#xD;
             within the preceding 4 weeks prior to study entry.&#xD;
&#xD;
          -  Patients with a history of non-compliance to medical regimens or who are considered&#xD;
             potentially unreliable or will not be able to complete the entire study. This includes&#xD;
             those in foster care, or those unable to keep follow-up appointments, maintain close&#xD;
             contact with the Principal Investigator, or complete all necessary studies to maintain&#xD;
             safety.&#xD;
&#xD;
          -  Pregnant or nursing (lactating) women, where pregnancy is defined as the state of the&#xD;
             female after conception and until the termination of gestation, confirmed by a&#xD;
             positive hCG laboratory test.&#xD;
      </textblock>
    </criteria>
    <gender>All</gender>
    <minimum_age>3 Years</minimum_age>
    <maximum_age>50 Years</maximum_age>
    <healthy_volunteers>No</healthy_volunteers>
  </eligibility>
  <overall_official>
    <last_name>Anne M Comi, MD</last_name>
    <role>Principal Investigator</role>
    <affiliation>Hugo W. Moser Research Institute at Kennedy Krieger, Inc.</affiliation>
  </overall_official>
  <location>
    <facility>
      <name>Kennedy Krieger Institute</name>
      <address>
        <city>Baltimore</city>
        <state>Maryland</state>
        <zip>21205</zip>
        <country>United States</country>
      </address>
    </facility>
  </location>
  <location_countries>
    <country>United States</country>
  </location_countries>
  <link>
    <url>https://www.kennedykrieger.org/patient-care/centers-and-programs/sturge-weber-syndrome-center</url>
    <description>Hunter Nelson Sturge-Weber Center Website</description>
  </link>
  <verification_date>May 2023</verification_date>
  <study_first_submitted>October 10, 2019</study_first_submitted>
  <study_first_submitted_qc>June 22, 2020</study_first_submitted_qc>
  <study_first_posted type="Actual">June 25, 2020</study_first_posted>
  <results_first_submitted>January 19, 2023</results_first_submitted>
  <results_first_submitted_qc>May 2, 2023</results_first_submitted_qc>
  <results_first_posted type="Actual">May 25, 2023</results_first_posted>
  <last_update_submitted>May 2, 2023</last_update_submitted>
  <last_update_submitted_qc>May 2, 2023</last_update_submitted_qc>
  <last_update_posted type="Actual">May 25, 2023</last_update_posted>
  <responsible_party>
    <responsible_party_type>Sponsor-Investigator</responsible_party_type>
    <investigator_affiliation>Hugo W. Moser Research Institute at Kennedy Krieger, Inc.</investigator_affiliation>
    <investigator_full_name>Anne Comi, MD</investigator_full_name>
    <investigator_title>Principal Investigator, Director Sturge-Weber Center, Kennedy Krieger Institute, Professor Johns Hopkins University School of Medicine</investigator_title>
  </responsible_party>
  <keyword>Sturge-Weber Syndrome</keyword>
  <keyword>SWS</keyword>
  <keyword>Cognitive impairments</keyword>
  <condition_browse>
    <!-- CAUTION:  The following MeSH terms are assigned with an imperfect algorithm            -->
    <mesh_term>Sturge-Weber Syndrome</mesh_term>
    <mesh_term>Brain Stem Infarctions</mesh_term>
    <mesh_term>Klippel-Trenaunay-Weber Syndrome</mesh_term>
    <mesh_term>Syndrome</mesh_term>
  </condition_browse>
  <intervention_browse>
    <!-- CAUTION:  The following MeSH terms are assigned with an imperfect algorithm            -->
    <mesh_term>Cannabidiol</mesh_term>
  </intervention_browse>
  <provided_document_section>
    <provided_document>
      <document_type>Study Protocol and Statistical Analysis Plan</document_type>
      <document_has_protocol>Yes</document_has_protocol>
      <document_has_icf>No</document_has_icf>
      <document_has_sap>Yes</document_has_sap>
      <document_date>June 29, 2021</document_date>
      <document_url>https://ClinicalTrials.gov/ProvidedDocs/46/NCT04447846/Prot_SAP_000.pdf</document_url>
    </provided_document>
  </provided_document_section>

  <clinical_results>

    <participant_flow>
      <pre_assignment_details>After consent, subject 10 was not enrolled as they did not meet the inclusion criteria of remaining at a stable does of anti-epileptic drugs 4 weeks prior to enrollment.</pre_assignment_details>
      <group_list>
        <group group_id="P1">
          <title>Cannabidiol/ Epidiolex</title>
          <description>All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome.&#xD;
Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.</description>
        </group>
      </group_list>
      <period_list>
        <period>
          <title>Overall Study</title>
          <milestone_list>
            <milestone>
              <title>STARTED</title>
              <participants_list>
                <participants group_id="P1" count="10"/>
              </participants_list>
            </milestone>
            <milestone>
              <title>COMPLETED</title>
              <participants_list>
                <participants group_id="P1" count="9"/>
              </participants_list>
            </milestone>
            <milestone>
              <title>NOT COMPLETED</title>
              <participants_list>
                <participants group_id="P1" count="1"/>
              </participants_list>
            </milestone>
          </milestone_list>
          <drop_withdraw_reason_list>
            <drop_withdraw_reason>
              <title>Withdrew due to moderate side effects (fatigue)</title>
              <participants_list>
                <participants group_id="P1" count="1"/>
              </participants_list>
            </drop_withdraw_reason>
          </drop_withdraw_reason_list>
        </period>
      </period_list>
    </participant_flow>

    <baseline>
      <group_list>
        <group group_id="B1">
          <title>Cannabidiol</title>
          <description>Subjects took oral cannabidiol for 6 months (doses ranged from 5 mg/kg/day to 20 mg/kg/day). Neuropsychological, psychiatrics and motor assessments were administered at baseline and six months follow-up.</description>
        </group>
      </group_list>
      <analyzed_list>
        <analyzed>
          <units>Participants</units>
          <scope>Overall</scope>
          <count_list>
            <count group_id="B1" value="10"/>
          </count_list>
        </analyzed>
      </analyzed_list>
      <measure_list>
        <measure>
          <title>Age</title>
          <units>years</units>
          <param>Mean</param>
          <dispersion>Standard Deviation</dispersion>
          <class_list>
            <class>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="B1" value="13.83" spread="9.67"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
          </class_list>
        </measure>
        <measure>
          <title>Sex: Female, Male</title>
          <units>Participants</units>
          <param>Count of Participants</param>
          <class_list>
            <class>
              <category_list>
                <category>
                  <title>Female</title>
                  <measurement_list>
                    <measurement group_id="B1" value="6"/>
                  </measurement_list>
                </category>
                <category>
                  <title>Male</title>
                  <measurement_list>
                    <measurement group_id="B1" value="4"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
          </class_list>
        </measure>
        <measure>
          <title>Ethnicity (NIH/OMB)</title>
          <units>Participants</units>
          <param>Count of Participants</param>
          <class_list>
            <class>
              <category_list>
                <category>
                  <title>Hispanic or Latino</title>
                  <measurement_list>
                    <measurement group_id="B1" value="2"/>
                  </measurement_list>
                </category>
                <category>
                  <title>Not Hispanic or Latino</title>
                  <measurement_list>
                    <measurement group_id="B1" value="7"/>
                  </measurement_list>
                </category>
                <category>
                  <title>Unknown or Not Reported</title>
                  <measurement_list>
                    <measurement group_id="B1" value="1"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
          </class_list>
        </measure>
        <measure>
          <title>Race (NIH/OMB)</title>
          <units>Participants</units>
          <param>Count of Participants</param>
          <class_list>
            <class>
              <category_list>
                <category>
                  <title>American Indian or Alaska Native</title>
                  <measurement_list>
                    <measurement group_id="B1" value="0"/>
                  </measurement_list>
                </category>
                <category>
                  <title>Asian</title>
                  <measurement_list>
                    <measurement group_id="B1" value="0"/>
                  </measurement_list>
                </category>
                <category>
                  <title>Native Hawaiian or Other Pacific Islander</title>
                  <measurement_list>
                    <measurement group_id="B1" value="1"/>
                  </measurement_list>
                </category>
                <category>
                  <title>Black or African American</title>
                  <measurement_list>
                    <measurement group_id="B1" value="0"/>
                  </measurement_list>
                </category>
                <category>
                  <title>White</title>
                  <measurement_list>
                    <measurement group_id="B1" value="6"/>
                  </measurement_list>
                </category>
                <category>
                  <title>More than one race</title>
                  <measurement_list>
                    <measurement group_id="B1" value="2"/>
                  </measurement_list>
                </category>
                <category>
                  <title>Unknown or Not Reported</title>
                  <measurement_list>
                    <measurement group_id="B1" value="1"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
          </class_list>
        </measure>
        <measure>
          <title>Region of Enrollment</title>
          <units>participants</units>
          <param>Number</param>
          <class_list>
            <class>
              <title>United States</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="B1" value="10"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
          </class_list>
        </measure>
        <measure>
          <title>Brain Involvement</title>
          <units>Participants</units>
          <param>Count of Participants</param>
          <class_list>
            <class>
              <category_list>
                <category>
                  <title>Right-Sided Brain Involvement</title>
                  <measurement_list>
                    <measurement group_id="B1" value="6"/>
                  </measurement_list>
                </category>
                <category>
                  <title>Left-Sided Brain Involvement</title>
                  <measurement_list>
                    <measurement group_id="B1" value="2"/>
                  </measurement_list>
                </category>
                <category>
                  <title>Bilateral Brain Involvement</title>
                  <measurement_list>
                    <measurement group_id="B1" value="2"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
          </class_list>
        </measure>
        <measure>
          <title>Skin Involvement</title>
          <units>Participants</units>
          <param>Count of Participants</param>
          <class_list>
            <class>
              <category_list>
                <category>
                  <title>Right-Sided Skin Involvement</title>
                  <measurement_list>
                    <measurement group_id="B1" value="4"/>
                  </measurement_list>
                </category>
                <category>
                  <title>Left-Sided Skin Involvement</title>
                  <measurement_list>
                    <measurement group_id="B1" value="2"/>
                  </measurement_list>
                </category>
                <category>
                  <title>Bilateral Skin Involvement</title>
                  <measurement_list>
                    <measurement group_id="B1" value="4"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
          </class_list>
        </measure>
        <measure>
          <title>Eye Involvement</title>
          <units>Participants</units>
          <param>Count of Participants</param>
          <class_list>
            <class>
              <category_list>
                <category>
                  <title>Right-Sided Eye Involvement</title>
                  <measurement_list>
                    <measurement group_id="B1" value="4"/>
                  </measurement_list>
                </category>
                <category>
                  <title>Left-Sided Eye Involvement</title>
                  <measurement_list>
                    <measurement group_id="B1" value="2"/>
                  </measurement_list>
                </category>
                <category>
                  <title>Bilateral Eye Involvement</title>
                  <measurement_list>
                    <measurement group_id="B1" value="1"/>
                  </measurement_list>
                </category>
                <category>
                  <title>No Eye Involvement</title>
                  <measurement_list>
                    <measurement group_id="B1" value="3"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
          </class_list>
        </measure>
      </measure_list>
    </baseline>

    <outcome_list>
      <outcome>
        <type>Primary</type>
        <title>List Sorting Working Memory Test</title>
        <description>Data on cognitive function was collected using the List Sorting Working Memory Test from the NIH Toolbox. Data was collected on working memory performance, which was transformed into a t-score from 0 to 100 where a higher t-score indicates better performance. T-score of 50 indicates the population mean with a standard deviation of 10. Data was collected at baseline and after 6 months on study drug.</description>
        <time_frame>Baseline, Follow-up (6 months)</time_frame>
        <population>Subject 1 was unable to complete this assessment due to lack of understanding of the instructions. Subject 10 was unable to complete this assessment due to a system error, where the fully adjusted score was not obtained. Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.</population>
        <group_list>
          <group group_id="O1">
            <title>Cannabidiol/ Epidiolex</title>
            <description>All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome.&#xD;
Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.</description>
          </group>
        </group_list>
        <measure>
          <title>List Sorting Working Memory Test</title>
          <description>Data on cognitive function was collected using the List Sorting Working Memory Test from the NIH Toolbox. Data was collected on working memory performance, which was transformed into a t-score from 0 to 100 where a higher t-score indicates better performance. T-score of 50 indicates the population mean with a standard deviation of 10. Data was collected at baseline and after 6 months on study drug.</description>
          <population>Subject 1 was unable to complete this assessment due to lack of understanding of the instructions. Subject 10 was unable to complete this assessment due to a system error, where the fully adjusted score was not obtained. Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.</population>
          <units>T-score</units>
          <param>Mean</param>
          <dispersion>Standard Deviation</dispersion>
          <analyzed_list>
            <analyzed>
              <units>Participants</units>
              <scope>Measure</scope>
              <count_list>
                <count group_id="O1" value="7"/>
              </count_list>
            </analyzed>
          </analyzed_list>
          <class_list>
            <class>
              <title>Baseline List Sorting Working Memory Fully Adjusted Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="37.14" spread="10.92"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-Up List Sorting Working Memory Fully Adjusted Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="39.14" spread="13.78"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
          </class_list>
        </measure>
      </outcome>
      <outcome>
        <type>Secondary</type>
        <title>Picture Vocabulary Test</title>
        <description>Data on cognitive function was collected using the Picture Vocabulary subtest from the NIH Toolbox. Single words are presented via an audio file, paired simultaneously with 4 screen images of objects, actions, and/or depictions of concepts. The task is to pick the picture that matches the spoken word. Performance on the task was transformed into a t-score from 0 to 100 where a higher t-score indicates better performance. T-score of 50 indicates the population mean with a standard deviation of 10. Data was collected at baseline and after 6 months on study drug.</description>
        <time_frame>Baseline, Follow-up (6 months)</time_frame>
        <population>Subject 10 did not complete this assessment due to a technical issue where the fully adjusted score was not calculated. Subject 9 was withdrawn from the study prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.</population>
        <group_list>
          <group group_id="O1">
            <title>Cannabidiol/ Epidiolex</title>
            <description>All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome.&#xD;
Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.</description>
          </group>
        </group_list>
        <measure>
          <title>Picture Vocabulary Test</title>
          <description>Data on cognitive function was collected using the Picture Vocabulary subtest from the NIH Toolbox. Single words are presented via an audio file, paired simultaneously with 4 screen images of objects, actions, and/or depictions of concepts. The task is to pick the picture that matches the spoken word. Performance on the task was transformed into a t-score from 0 to 100 where a higher t-score indicates better performance. T-score of 50 indicates the population mean with a standard deviation of 10. Data was collected at baseline and after 6 months on study drug.</description>
          <population>Subject 10 did not complete this assessment due to a technical issue where the fully adjusted score was not calculated. Subject 9 was withdrawn from the study prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.</population>
          <units>T-score</units>
          <param>Mean</param>
          <dispersion>Standard Deviation</dispersion>
          <analyzed_list>
            <analyzed>
              <units>Participants</units>
              <scope>Measure</scope>
              <count_list>
                <count group_id="O1" value="8"/>
              </count_list>
            </analyzed>
          </analyzed_list>
          <class_list>
            <class>
              <title>Baseline Mean of the Fully Adjusted Picture Vocabulary Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="42.88" spread="8.86"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean of the Fully Adjusted Picture Vocabulary Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="42.38" spread="9.43"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
          </class_list>
        </measure>
      </outcome>
      <outcome>
        <type>Secondary</type>
        <title>Seizure Frequency</title>
        <description>Change in seizure frequency by seizure score at pre-treatment baseline and after six months. The seizure score is taken from the Sturge-Weber Neuroscore on a scale of 0 to 4 where 0=none, 1=1+. but controlled, 2=Breakthrough, 3=monthly, 4=Weekly+. Higher scores indicate worse outcome.</description>
        <time_frame>Baseline, Follow-up (6 months)</time_frame>
        <population>Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.</population>
        <group_list>
          <group group_id="O1">
            <title>Cannabidiol/ Epidiolex</title>
            <description>All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome.&#xD;
Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.</description>
          </group>
        </group_list>
        <measure>
          <title>Seizure Frequency</title>
          <description>Change in seizure frequency by seizure score at pre-treatment baseline and after six months. The seizure score is taken from the Sturge-Weber Neuroscore on a scale of 0 to 4 where 0=none, 1=1+. but controlled, 2=Breakthrough, 3=monthly, 4=Weekly+. Higher scores indicate worse outcome.</description>
          <population>Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.</population>
          <units>score on a scale</units>
          <param>Mean</param>
          <dispersion>Standard Deviation</dispersion>
          <analyzed_list>
            <analyzed>
              <units>Participants</units>
              <scope>Measure</scope>
              <count_list>
                <count group_id="O1" value="9"/>
              </count_list>
            </analyzed>
          </analyzed_list>
          <class_list>
            <class>
              <title>Baseline Seizure Score from Sturge-Weber Neuroscore</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="1.11" spread="0.33"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Seizure Score from Sturge-Weber Neuroscore</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="1.00" spread="0.00"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
          </class_list>
        </measure>
      </outcome>
      <outcome>
        <type>Secondary</type>
        <title>Migraine Severity</title>
        <description>Data on migraine severity will be collected using patient responses to questions on a standard six-point scale. Data will be collected on the frequency of an event (e.g. feelings of frustration, performance of daily activities) which will be transformed into a score from 0 to 100 where higher scores indicate a less migraine severity and better outcomes. Data will be collected at baseline and after 6 months on study drug.</description>
        <time_frame>Baseline, Follow-up (6 months)</time_frame>
        <population>Subject 7 misplaced this questionnaire and was not included in analysis. Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.</population>
        <group_list>
          <group group_id="O1">
            <title>Cannabidiol/ Epidiolex</title>
            <description>All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome.&#xD;
Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.</description>
          </group>
        </group_list>
        <measure>
          <title>Migraine Severity</title>
          <description>Data on migraine severity will be collected using patient responses to questions on a standard six-point scale. Data will be collected on the frequency of an event (e.g. feelings of frustration, performance of daily activities) which will be transformed into a score from 0 to 100 where higher scores indicate a less migraine severity and better outcomes. Data will be collected at baseline and after 6 months on study drug.</description>
          <population>Subject 7 misplaced this questionnaire and was not included in analysis. Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.</population>
          <units>score on a scale</units>
          <param>Mean</param>
          <dispersion>Standard Deviation</dispersion>
          <analyzed_list>
            <analyzed>
              <units>Participants</units>
              <scope>Measure</scope>
              <count_list>
                <count group_id="O1" value="8"/>
              </count_list>
            </analyzed>
          </analyzed_list>
          <class_list>
            <class>
              <title>Baseline Mean Migraine-Quality of Life Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="96.83" spread="7.62"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean Migraine-Quality of Life Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="97.50" spread="4.69"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
          </class_list>
        </measure>
      </outcome>
      <outcome>
        <type>Secondary</type>
        <title>Modified House Classification Scores</title>
        <description>Data on motor function was collected using a Modified House Classification. Data was collected on the ability to complete a task with the subject's non-dominant hand which was transformed into a score from 1 to 8 and 0 to 32 where higher scores indicate better motor function. Data was collected at baseline and after 6 months on study drug.</description>
        <time_frame>Baseline, Follow-up (6 months)</time_frame>
        <population>Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.</population>
        <group_list>
          <group group_id="O1">
            <title>Cannabidiol/ Epidiolex</title>
            <description>All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome.&#xD;
Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.</description>
          </group>
        </group_list>
        <measure>
          <title>Modified House Classification Scores</title>
          <description>Data on motor function was collected using a Modified House Classification. Data was collected on the ability to complete a task with the subject's non-dominant hand which was transformed into a score from 1 to 8 and 0 to 32 where higher scores indicate better motor function. Data was collected at baseline and after 6 months on study drug.</description>
          <population>Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.</population>
          <units>score on a scale</units>
          <param>Mean</param>
          <dispersion>Standard Deviation</dispersion>
          <analyzed_list>
            <analyzed>
              <units>Participants</units>
              <scope>Measure</scope>
              <count_list>
                <count group_id="O1" value="9"/>
              </count_list>
            </analyzed>
          </analyzed_list>
          <class_list>
            <class>
              <title>Baseline Mean Highest Category in Which All Items were Successfully Completed</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="5.11" spread="0.93"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean Highest Category in Which All Items were Successfully Completed</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="5.44" spread="1.33"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Total Number of Items Successfully Completed</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="26.67" spread="4.12"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean Total Number of Items Successfully Completed</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="27.44" spread="4.75"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
          </class_list>
        </measure>
      </outcome>
      <outcome>
        <type>Secondary</type>
        <title>Erhardt Developmental Prehension Assessment Scores</title>
        <description>Data on motor function was collected using the Erhardt Developmental Prehension assessment. Data was collected on the ability to complete a task with each hand, which was scored as age equivalence of task performance (in months). Higher scores indicate better motor function. Data was collected at baseline and after 6 months on study drug.</description>
        <time_frame>Baseline, Follow-up (6 months)</time_frame>
        <population>Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.</population>
        <group_list>
          <group group_id="O1">
            <title>Cannabidiol/ Epidiolex</title>
            <description>All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome.&#xD;
Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.</description>
          </group>
        </group_list>
        <measure>
          <title>Erhardt Developmental Prehension Assessment Scores</title>
          <description>Data on motor function was collected using the Erhardt Developmental Prehension assessment. Data was collected on the ability to complete a task with each hand, which was scored as age equivalence of task performance (in months). Higher scores indicate better motor function. Data was collected at baseline and after 6 months on study drug.</description>
          <population>Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.</population>
          <units>Months</units>
          <param>Mean</param>
          <dispersion>Standard Deviation</dispersion>
          <analyzed_list>
            <analyzed>
              <units>Participants</units>
              <scope>Measure</scope>
              <count_list>
                <count group_id="O1" value="9"/>
              </count_list>
            </analyzed>
          </analyzed_list>
          <class_list>
            <class>
              <title>Baseline Mean Dominant Hand Total Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="54.22" spread="2.64"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean Dominant Hand Total Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="53.56" spread="4.04"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Non-Dominant Hand Total Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="51.22" spread="9.48"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean Non-Dominant Hand Total Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="53.78" spread="4.15"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
          </class_list>
        </measure>
      </outcome>
      <outcome>
        <type>Secondary</type>
        <title>Pediatric Evaluation of Disability Inventory Computer Adapted Test</title>
        <description>Data on motor function was collected using the Pediatric Evaluation of Disability Inventory Computer Adapted Test. Data was collected on the subject's ability to complete tasks involved in daily activities, mobility, and social/ cognitive activities. The data was transformed into scaled scores ranging from 20 to 80, where higher scores indicate better motor function. Data was collected at baseline and after 6 months on study drug.</description>
        <time_frame>Baseline, Follow-up (6 months)</time_frame>
        <population>Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.</population>
        <group_list>
          <group group_id="O1">
            <title>Cannabidiol/ Epidiolex</title>
            <description>All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome.&#xD;
Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.</description>
          </group>
        </group_list>
        <measure>
          <title>Pediatric Evaluation of Disability Inventory Computer Adapted Test</title>
          <description>Data on motor function was collected using the Pediatric Evaluation of Disability Inventory Computer Adapted Test. Data was collected on the subject's ability to complete tasks involved in daily activities, mobility, and social/ cognitive activities. The data was transformed into scaled scores ranging from 20 to 80, where higher scores indicate better motor function. Data was collected at baseline and after 6 months on study drug.</description>
          <population>Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.</population>
          <units>score on a scale</units>
          <param>Mean</param>
          <dispersion>Standard Deviation</dispersion>
          <analyzed_list>
            <analyzed>
              <units>Participants</units>
              <scope>Measure</scope>
              <count_list>
                <count group_id="O1" value="9"/>
              </count_list>
            </analyzed>
          </analyzed_list>
          <class_list>
            <class>
              <title>Baseline Mean Score for Daily Activities</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="58.67" spread="7.76"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean Score for Daily Activities</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="60.00" spread="6.50"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Score for Mobility</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="67.11" spread="4.76"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-Up Mean Score for Mobility</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="68.56" spread="4.07"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Score for Responsibility</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="52.44" spread="11.67"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-Up Mean Score for Responsibility</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="53.89" spread="11.10"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Score for Social Cognitive</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="66.89" spread="5.35"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean Score for Social Cognitive</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="67.22" spread="4.63"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
          </class_list>
        </measure>
      </outcome>
      <outcome>
        <type>Secondary</type>
        <title>ABILHAND Questionnaire</title>
        <description>Data on motor function was collected using the ABILHAND questionnaire, a measure of manual ability for adults with upper limb impairments. Data was collected on the subject's ability to complete daily activities that involve the upper limbs. Score was collected as a patient measure with scores ranging from -10 to 10. Higher scores indicate better motor function. Data was collected at baseline and after 6 months on study drug.</description>
        <time_frame>Baseline, Follow-up (6 months)</time_frame>
        <population>Subject 7 misplaced this questionnaire and was not included in analysis. Subject 9 was withdrawn prior to the 6 month follow-up visit due to persistent side effect and was not included in analysis.</population>
        <group_list>
          <group group_id="O1">
            <title>Cannabidiol/ Epidiolex</title>
            <description>All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome.&#xD;
Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.</description>
          </group>
        </group_list>
        <measure>
          <title>ABILHAND Questionnaire</title>
          <description>Data on motor function was collected using the ABILHAND questionnaire, a measure of manual ability for adults with upper limb impairments. Data was collected on the subject's ability to complete daily activities that involve the upper limbs. Score was collected as a patient measure with scores ranging from -10 to 10. Higher scores indicate better motor function. Data was collected at baseline and after 6 months on study drug.</description>
          <population>Subject 7 misplaced this questionnaire and was not included in analysis. Subject 9 was withdrawn prior to the 6 month follow-up visit due to persistent side effect and was not included in analysis.</population>
          <units>score on a scale</units>
          <param>Mean</param>
          <dispersion>Standard Deviation</dispersion>
          <analyzed_list>
            <analyzed>
              <units>Participants</units>
              <scope>Measure</scope>
              <count_list>
                <count group_id="O1" value="8"/>
              </count_list>
            </analyzed>
          </analyzed_list>
          <class_list>
            <class>
              <title>Baseline Mean of the ABILHAND</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="1.81" spread="1.98"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean of the ABILHAND</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="2.88" spread="1.39"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
          </class_list>
        </measure>
      </outcome>
      <outcome>
        <type>Secondary</type>
        <title>Wechsler Intelligence Scale for Children, Fifth Edition (WISC-V) or Wechsler Adult Intelligence Scale (WAIS-IV)</title>
        <description>Data on cognitive function was collected using selected subtests, from either the Wechsler Intelligence Scale for Children, Fifth Edition (WISC-V) or the Wechsler Adult Intelligence Scale (WAIS-IV). For the WISC-V and WAIS-IV subtests selected, scaled scores were derived from the normative data which account for age and demographic information. The selected WISC-V/WAIS-IV subtests were as follows: Digit Span, Symbol Search, Coding and Processing Speed; the first three subtests were scored from 0-10, the fourth subtest from 0-100, and for all subtests higher score is better. The selected subtests, from the WISC-V and the WAIS-IV, were combined to increase statistical power.&#xD;
Statistically rare changes in individual test scores were determined using a reliable change index methodology based upon normative information for these assessments.</description>
        <time_frame>Baseline, Follow-up (6 months)</time_frame>
        <population>Subjects 1 and 10 did not complete this questionnaire as they were too young. Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.</population>
        <group_list>
          <group group_id="O1">
            <title>Cannabidiol/ Epidiolex</title>
            <description>All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome.&#xD;
Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.</description>
          </group>
        </group_list>
        <measure>
          <title>Wechsler Intelligence Scale for Children, Fifth Edition (WISC-V) or Wechsler Adult Intelligence Scale (WAIS-IV)</title>
          <description>Data on cognitive function was collected using selected subtests, from either the Wechsler Intelligence Scale for Children, Fifth Edition (WISC-V) or the Wechsler Adult Intelligence Scale (WAIS-IV). For the WISC-V and WAIS-IV subtests selected, scaled scores were derived from the normative data which account for age and demographic information. The selected WISC-V/WAIS-IV subtests were as follows: Digit Span, Symbol Search, Coding and Processing Speed; the first three subtests were scored from 0-10, the fourth subtest from 0-100, and for all subtests higher score is better. The selected subtests, from the WISC-V and the WAIS-IV, were combined to increase statistical power.&#xD;
Statistically rare changes in individual test scores were determined using a reliable change index methodology based upon normative information for these assessments.</description>
          <population>Subjects 1 and 10 did not complete this questionnaire as they were too young. Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.</population>
          <units>score on a scale</units>
          <param>Mean</param>
          <dispersion>Standard Deviation</dispersion>
          <analyzed_list>
            <analyzed>
              <units>Participants</units>
              <scope>Measure</scope>
              <count_list>
                <count group_id="O1" value="7"/>
              </count_list>
            </analyzed>
          </analyzed_list>
          <class_list>
            <class>
              <title>Baseline Mean Digit Span Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="5.71" spread="2.81"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean Digit Span Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="6.43" spread="2.07"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Symbol Search Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="6.00" spread="2.71"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean Symbol Search Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="5.57" spread="2.07"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Coding Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="5.57" spread="2.88"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean Coding Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="4.71" spread="2.22"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Processing Speed Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="77.14" spread="13.93"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean Processing Speed Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="73.43" spread="11.19"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
          </class_list>
        </measure>
      </outcome>
      <outcome>
        <type>Secondary</type>
        <title>Pediatric Neurological Quality of Life (Neuro-QoL)</title>
        <description>Data on cognitive function was collected using Neurological Quality of Life scales from the NIH Toolbox. Data on frequency of an event (e.g. forgetting schoolwork) was collected and transformed into a t-score from 0 to 100 where higher t-scores indicate worse anger, worse anxiety, worse pain, better social relationships, worse stigma, worse depression, better cognitive function, and worse fatigue. T-score of 50 indicates the population mean with a standard deviation of 10. Data was collected at baseline and 6 months on study drug.</description>
        <time_frame>Baseline, Follow-up (6 months)</time_frame>
        <population>Subjects below the age of 18 completed the pediatric version of this questionnaire and were included in analysis.</population>
        <group_list>
          <group group_id="O1">
            <title>Cannabidiol/ Epidiolex</title>
            <description>All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome.&#xD;
Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.</description>
          </group>
        </group_list>
        <measure>
          <title>Pediatric Neurological Quality of Life (Neuro-QoL)</title>
          <description>Data on cognitive function was collected using Neurological Quality of Life scales from the NIH Toolbox. Data on frequency of an event (e.g. forgetting schoolwork) was collected and transformed into a t-score from 0 to 100 where higher t-scores indicate worse anger, worse anxiety, worse pain, better social relationships, worse stigma, worse depression, better cognitive function, and worse fatigue. T-score of 50 indicates the population mean with a standard deviation of 10. Data was collected at baseline and 6 months on study drug.</description>
          <population>Subjects below the age of 18 completed the pediatric version of this questionnaire and were included in analysis.</population>
          <units>T-score</units>
          <param>Mean</param>
          <dispersion>Standard Deviation</dispersion>
          <analyzed_list>
            <analyzed>
              <units>Participants</units>
              <scope>Measure</scope>
              <count_list>
                <count group_id="O1" value="6"/>
              </count_list>
            </analyzed>
          </analyzed_list>
          <class_list>
            <class>
              <title>Baseline Mean Pediatric Score for Anger</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="53.02" spread="3.09"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean Pediatric Score for Anger</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="49.67" spread="7.00"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Pediatric Score for Anxiety</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="45.20" spread="5.79"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean Pediatric Score for Anxiety</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="41.07" spread="5.74"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Pediatric Score for Pain</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="40.20" spread="4.16"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean Pediatric Score for Pain</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="43.50" spread="5.89"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Pediatric Score for Social Relations</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="43.28" spread="15.10"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean Pediatric Score for Social Relations</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="54.33" spread="6.96"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Pediatric Score for Stigma</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="42.45" spread="6.42"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean Pediatric Score for Stigma</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="44.52" spread="4.88"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Pediatric Score for Depression</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="44.07" spread="6.29"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean Pediatric Score for Depression</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="44.62" spread="8.58"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Pediatric Score for Cognitive Function</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="49.27" spread="3.36"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean Pediatric Score for Cognitive Function</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="51.33" spread="6.44"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Pediatric Score for Fatigue</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="46.38" spread="9.44"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean Pediatric Score for Fatigue</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="45.97" spread="8.14"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
          </class_list>
        </measure>
      </outcome>
      <outcome>
        <type>Secondary</type>
        <title>Behavior Rating Inventory of Executive Function, Second Edition (BRIEF-2)</title>
        <description>Data on executive function was collected using the Behavior Rating Inventory of Executive Function, Second Edition (BRIEF-2). Data on the frequency of an event (e.g. becomes upset too easily) was collected and transformed into a t-score. The following BRIEF-2 subtests were evaluated: Behavioral Regulation Index, Emotional Regulation Index, Cognitive Regulation Index, and Global Executive Composite. For each BRIEF-2 subtest, t-scores range from 0 to 100; a score of 50 indicates the population mean with a standard deviation of 10. For all BASC-3 subtests, higher scores are worse outcome.</description>
        <time_frame>Baseline, Follow-up (6 months)</time_frame>
        <population>Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.</population>
        <group_list>
          <group group_id="O1">
            <title>Cannabidiol/ Epidiolex</title>
            <description>All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome.&#xD;
Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.</description>
          </group>
        </group_list>
        <measure>
          <title>Behavior Rating Inventory of Executive Function, Second Edition (BRIEF-2)</title>
          <description>Data on executive function was collected using the Behavior Rating Inventory of Executive Function, Second Edition (BRIEF-2). Data on the frequency of an event (e.g. becomes upset too easily) was collected and transformed into a t-score. The following BRIEF-2 subtests were evaluated: Behavioral Regulation Index, Emotional Regulation Index, Cognitive Regulation Index, and Global Executive Composite. For each BRIEF-2 subtest, t-scores range from 0 to 100; a score of 50 indicates the population mean with a standard deviation of 10. For all BASC-3 subtests, higher scores are worse outcome.</description>
          <population>Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.</population>
          <units>T-score</units>
          <param>Mean</param>
          <dispersion>Standard Deviation</dispersion>
          <analyzed_list>
            <analyzed>
              <units>Participants</units>
              <scope>Measure</scope>
              <count_list>
                <count group_id="O1" value="9"/>
              </count_list>
            </analyzed>
          </analyzed_list>
          <class_list>
            <class>
              <title>Baseline Mean for Behavioral Regulation Index</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="55.11" spread="11.66"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-Up Mean for Behavioral Regulation Index</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="52.44" spread="13.60"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean for Emotional Regulation Index</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="60.22" spread="10.24"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean for Emotional Regulation Index</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="51.22" spread="12.29"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean for Cognitive Regulation Index</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="54.11" spread="7.06"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean for Cognitive Regulation Index</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="54.22" spread="10.59"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean for Global Executive Composite</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="56.78" spread="8.17"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean for Global Executive Composite</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="53.33" spread="11.57"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
          </class_list>
        </measure>
      </outcome>
      <outcome>
        <type>Secondary</type>
        <title>Social Responsiveness Scale, Second Edition (SRS-2)</title>
        <description>Data on social function was collected using the Social Responsiveness Scale-Second Edition (SRS-2). Data on a child's ability to engage in emotionally appropriate reciprocal social interactions in naturalistic settings was collected and transformed into a t-score from 0 to 100 where higher scores indicate greater impairment in social function. T-score of 50 indicates the population mean with a standard deviation of 10. Data was collected at baseline and 6 months on study drug.</description>
        <time_frame>Baseline, Follow-up (6 months)</time_frame>
        <population>Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.</population>
        <group_list>
          <group group_id="O1">
            <title>Cannabidiol/ Epidiolex</title>
            <description>All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome.&#xD;
Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.</description>
          </group>
        </group_list>
        <measure>
          <title>Social Responsiveness Scale, Second Edition (SRS-2)</title>
          <description>Data on social function was collected using the Social Responsiveness Scale-Second Edition (SRS-2). Data on a child's ability to engage in emotionally appropriate reciprocal social interactions in naturalistic settings was collected and transformed into a t-score from 0 to 100 where higher scores indicate greater impairment in social function. T-score of 50 indicates the population mean with a standard deviation of 10. Data was collected at baseline and 6 months on study drug.</description>
          <population>Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.</population>
          <units>T-score</units>
          <param>Mean</param>
          <dispersion>Standard Deviation</dispersion>
          <analyzed_list>
            <analyzed>
              <units>Participants</units>
              <scope>Measure</scope>
              <count_list>
                <count group_id="O1" value="9"/>
              </count_list>
            </analyzed>
          </analyzed_list>
          <class_list>
            <class>
              <title>Baseline Mean Total Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="54.22" spread="7.71"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean Total Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="49.22" spread="7.09"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
          </class_list>
        </measure>
      </outcome>
      <outcome>
        <type>Secondary</type>
        <title>Behavioral Assessment System for Children, Third Edition (BASC-3)</title>
        <description>Data on behavioral function was collected using the Behavioral Assessment System for Children, Third Edition (BASC-3). Data on the frequency of a behavior (e.g. avoids eye contact) was collected and transformed into a t-score. Subscales for the BASC-3 scored were: External Problems Composite, the Internal Problems Composite, the Behavioral Symptoms Index, and the Adaptive Skills Composite. For the first three subscales, lower t-score indicates better outcome; for the fourth, a lower t-score indicates worse outcome. T-scores for all the BASC-3 subscales range from 0 to 100, and a t-score of 50 indicates the population mean with a standard deviation of 10.</description>
        <time_frame>Baseline, Follow-up (6 months)</time_frame>
        <population>Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.</population>
        <group_list>
          <group group_id="O1">
            <title>Cannabidiol/ Epidiolex</title>
            <description>All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome.&#xD;
Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.</description>
          </group>
        </group_list>
        <measure>
          <title>Behavioral Assessment System for Children, Third Edition (BASC-3)</title>
          <description>Data on behavioral function was collected using the Behavioral Assessment System for Children, Third Edition (BASC-3). Data on the frequency of a behavior (e.g. avoids eye contact) was collected and transformed into a t-score. Subscales for the BASC-3 scored were: External Problems Composite, the Internal Problems Composite, the Behavioral Symptoms Index, and the Adaptive Skills Composite. For the first three subscales, lower t-score indicates better outcome; for the fourth, a lower t-score indicates worse outcome. T-scores for all the BASC-3 subscales range from 0 to 100, and a t-score of 50 indicates the population mean with a standard deviation of 10.</description>
          <population>Subject 9 was withdrawn prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.</population>
          <units>T-score</units>
          <param>Mean</param>
          <dispersion>Standard Deviation</dispersion>
          <analyzed_list>
            <analyzed>
              <units>Participants</units>
              <scope>Measure</scope>
              <count_list>
                <count group_id="O1" value="9"/>
              </count_list>
            </analyzed>
          </analyzed_list>
          <class_list>
            <class>
              <title>Baseline Mean External Problems Composite</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="51.22" spread="6.10"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean External Problems Composite</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="50.11" spread="6.31"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Internal Problems Composite</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="52.22" spread="5.81"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-Up Mean Internal Problems Composite</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="46.22" spread="7.17"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Behavioral Symptoms Index</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="54.22" spread="4.21"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean Behavioral Symptoms Index</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="50.11" spread="8.19"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Adaptive Skills Composite</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="45.00" spread="8.29"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-Up Mean Adaptive Skills Composite</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="48.44" spread="7.63"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
          </class_list>
        </measure>
      </outcome>
      <outcome>
        <type>Secondary</type>
        <title>Screen for Child Anxiety Related Disorders (SCARED)</title>
        <description>Data on anxiety was collected using the Screen for Child Anxiety Related Disorders (SCARED). Data on the truthfulness of a statement (e.g. I am nervous) was collected and transformed into a score from 0 to 82 where higher scores indicate greater anxiety. Data was collected at baseline and 6 months on study drug.</description>
        <time_frame>Baseline, Follow-up (6 months)</time_frame>
        <population>Subject 7 misplaced this assessment and was not included in analysis. Subject 9 was withdrawn from the study prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.</population>
        <group_list>
          <group group_id="O1">
            <title>Cannabidiol/ Epidiolex</title>
            <description>All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome.&#xD;
Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.</description>
          </group>
        </group_list>
        <measure>
          <title>Screen for Child Anxiety Related Disorders (SCARED)</title>
          <description>Data on anxiety was collected using the Screen for Child Anxiety Related Disorders (SCARED). Data on the truthfulness of a statement (e.g. I am nervous) was collected and transformed into a score from 0 to 82 where higher scores indicate greater anxiety. Data was collected at baseline and 6 months on study drug.</description>
          <population>Subject 7 misplaced this assessment and was not included in analysis. Subject 9 was withdrawn from the study prior to the 6 month follow-up visit due to a persistent side effect and was not included in analysis.</population>
          <units>score on a scale</units>
          <param>Mean</param>
          <dispersion>Standard Deviation</dispersion>
          <analyzed_list>
            <analyzed>
              <units>Participants</units>
              <scope>Measure</scope>
              <count_list>
                <count group_id="O1" value="8"/>
              </count_list>
            </analyzed>
          </analyzed_list>
          <class_list>
            <class>
              <title>Baseline Mean of Total Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="14.86" spread="14.49"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean of Total Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="11.57" spread="11.16"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
          </class_list>
        </measure>
      </outcome>
      <outcome>
        <type>Secondary</type>
        <title>Quality of Life in Childhood Epilepsy Questionnaire (QOLCE-55)</title>
        <description>Data on quality of life was collected using the Quality of Life in Childhood Epilepsy Questionnaire (QOLCE-55). Data on the frequency of an event (e.g. had trouble concentrating on a task) was collected and transformed into a score from 0 to 100 where higher scores reflect better quality of life. Data was collected at baseline and 6 months on study drug.</description>
        <time_frame>Baseline, Follow-up (6 months)</time_frame>
        <population>Subject 7 misplaced this assessment and was not included in analysis. Subject 9 was withdrawn prior to the 6 month follow-up appointment due to a persistent side effect and was not included in analysis.</population>
        <group_list>
          <group group_id="O1">
            <title>Cannabidiol/ Epidiolex</title>
            <description>All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome.&#xD;
Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.</description>
          </group>
        </group_list>
        <measure>
          <title>Quality of Life in Childhood Epilepsy Questionnaire (QOLCE-55)</title>
          <description>Data on quality of life was collected using the Quality of Life in Childhood Epilepsy Questionnaire (QOLCE-55). Data on the frequency of an event (e.g. had trouble concentrating on a task) was collected and transformed into a score from 0 to 100 where higher scores reflect better quality of life. Data was collected at baseline and 6 months on study drug.</description>
          <population>Subject 7 misplaced this assessment and was not included in analysis. Subject 9 was withdrawn prior to the 6 month follow-up appointment due to a persistent side effect and was not included in analysis.</population>
          <units>score on a scale</units>
          <param>Mean</param>
          <dispersion>Standard Deviation</dispersion>
          <analyzed_list>
            <analyzed>
              <units>Participants</units>
              <scope>Measure</scope>
              <count_list>
                <count group_id="O1" value="8"/>
              </count_list>
            </analyzed>
          </analyzed_list>
          <class_list>
            <class>
              <title>Baseline Mean Total Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="61.17" spread="18.14"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean Total Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="71.39" spread="10.75"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
          </class_list>
        </measure>
      </outcome>
      <outcome>
        <type>Secondary</type>
        <title>Safety of Epidiolex</title>
        <description>Safety of Epidiolex was measured by the number of adverse events and serious adverse events that result from study drug.</description>
        <time_frame>Baseline, Follow-up (6 months)</time_frame>
        <group_list>
          <group group_id="O1">
            <title>Cannabidiol/ Epidiolex</title>
            <description>All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome.&#xD;
Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.</description>
          </group>
        </group_list>
        <measure>
          <title>Safety of Epidiolex</title>
          <description>Safety of Epidiolex was measured by the number of adverse events and serious adverse events that result from study drug.</description>
          <units>Events</units>
          <param>Number</param>
          <analyzed_list>
            <analyzed>
              <units>Participants</units>
              <scope>Measure</scope>
              <count_list>
                <count group_id="O1" value="10"/>
              </count_list>
            </analyzed>
          </analyzed_list>
          <class_list>
            <class>
              <title>Number of Adverse Events that Resulted from the Study Drug</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="24"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Number of Serious Adverse Events that Resulted from the Study Drug</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="0"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
          </class_list>
        </measure>
      </outcome>
      <outcome>
        <type>Secondary</type>
        <title>Neuroscore</title>
        <description>Data on neurological function was collected using the Neuroscore. Data on frequency of seizures, extent of hemiparesis, assessment of visual field cut, and degree of cognitive functioning was transformed into a score from 0 to 15 where higher scores indicate worse neurologic function. Data was collected at baseline and 6 months on study drug.</description>
        <time_frame>Baseline, Follow-up (6 months)</time_frame>
        <population>Subject 9 was withdrawn prior to the 6 month follow-up visit and not included in analysis.</population>
        <group_list>
          <group group_id="O1">
            <title>Cannabidiol/ Epidiolex</title>
            <description>All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome.&#xD;
Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.</description>
          </group>
        </group_list>
        <measure>
          <title>Neuroscore</title>
          <description>Data on neurological function was collected using the Neuroscore. Data on frequency of seizures, extent of hemiparesis, assessment of visual field cut, and degree of cognitive functioning was transformed into a score from 0 to 15 where higher scores indicate worse neurologic function. Data was collected at baseline and 6 months on study drug.</description>
          <population>Subject 9 was withdrawn prior to the 6 month follow-up visit and not included in analysis.</population>
          <units>score on a scale</units>
          <param>Mean</param>
          <dispersion>Standard Deviation</dispersion>
          <analyzed_list>
            <analyzed>
              <units>Participants</units>
              <scope>Measure</scope>
              <count_list>
                <count group_id="O1" value="9"/>
              </count_list>
            </analyzed>
          </analyzed_list>
          <class_list>
            <class>
              <title>Baseline Mean Seizure Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="1.11" spread="0.33"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-Up Mean Seizure Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="1.00" spread="0.00"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Hemiparesis Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="1.44" spread="1.13"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-Up Mean Hemiparesis Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="1.00" spread="0.87"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Visual Field Cut Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="0.11" spread="0.33"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-Up Mean Visual Field Cut Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="0.00" spread="0.00"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Cognitive Function Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="2.33" spread="0.50"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean Cognitive Function Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="1.44" spread="0.53"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Composite Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="5.00" spread="1.32"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean Composite Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="3.44" spread="0.88"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
          </class_list>
        </measure>
      </outcome>
      <outcome>
        <type>Secondary</type>
        <title>Port-wine Birthmark Score</title>
        <description>Data on facial port-wine birthmarks was collected using the Port-wine Birthmark Score. Data on percent of face covered, thickness of birthmark, and darkness of birthmark color was collected and transformed into a score from 0 to 43 where higher scores indicate greater severity and greater surface area involved. Data was collected at baseline and 6 months on study drug.</description>
        <time_frame>Baseline, Follow-up (6 months)</time_frame>
        <population>Subject 9 was withdrawn prior to the 6 month follow-up appointment and was not included in analysis.</population>
        <group_list>
          <group group_id="O1">
            <title>Cannabidiol/ Epidiolex</title>
            <description>All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome.&#xD;
Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.</description>
          </group>
        </group_list>
        <measure>
          <title>Port-wine Birthmark Score</title>
          <description>Data on facial port-wine birthmarks was collected using the Port-wine Birthmark Score. Data on percent of face covered, thickness of birthmark, and darkness of birthmark color was collected and transformed into a score from 0 to 43 where higher scores indicate greater severity and greater surface area involved. Data was collected at baseline and 6 months on study drug.</description>
          <population>Subject 9 was withdrawn prior to the 6 month follow-up appointment and was not included in analysis.</population>
          <units>score on a scale</units>
          <param>Mean</param>
          <dispersion>Standard Deviation</dispersion>
          <analyzed_list>
            <analyzed>
              <units>Participants</units>
              <scope>Measure</scope>
              <count_list>
                <count group_id="O1" value="9"/>
              </count_list>
            </analyzed>
          </analyzed_list>
          <class_list>
            <class>
              <title>Baseline Mean Total Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="8.56" spread="3.09"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-up Mean Total Score</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="8.11" spread="3.18"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
          </class_list>
        </measure>
      </outcome>
      <outcome>
        <type>Secondary</type>
        <title>Adult Neurological Quality of Life (Neuro-QoL)</title>
        <description>Data on cognitive function was collected using Neurological Quality of Life scales from the NIH Toolbox. Data on frequency of an event was collected and transformed into a t-score from 0 to 100. Higher t-scores indicate better communication, better ability to participate in social activity, worse anxiety, worse depression, worse emotional and behavioral dyscontrol, worse fatigue, better positive affect, worse sleep disturbance, better lower and upper extremity functions, worse stigma, better satisfaction with social roles, and better cognitive function. T-score of 50 indicates the population mean with a standard deviation of 10. Data was collected at baseline and 6 months on study drug.</description>
        <time_frame>Baseline, Follow-up (6 months)</time_frame>
        <population>Subjects above the age of 18 completed the pediatric version of this questionnaire and were included in analysis.</population>
        <group_list>
          <group group_id="O1">
            <title>Cannabidiol/ Epidiolex</title>
            <description>All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome.&#xD;
Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.</description>
          </group>
        </group_list>
        <measure>
          <title>Adult Neurological Quality of Life (Neuro-QoL)</title>
          <description>Data on cognitive function was collected using Neurological Quality of Life scales from the NIH Toolbox. Data on frequency of an event was collected and transformed into a t-score from 0 to 100. Higher t-scores indicate better communication, better ability to participate in social activity, worse anxiety, worse depression, worse emotional and behavioral dyscontrol, worse fatigue, better positive affect, worse sleep disturbance, better lower and upper extremity functions, worse stigma, better satisfaction with social roles, and better cognitive function. T-score of 50 indicates the population mean with a standard deviation of 10. Data was collected at baseline and 6 months on study drug.</description>
          <population>Subjects above the age of 18 completed the pediatric version of this questionnaire and were included in analysis.</population>
          <units>T-score</units>
          <param>Mean</param>
          <dispersion>Standard Deviation</dispersion>
          <analyzed_list>
            <analyzed>
              <units>Participants</units>
              <scope>Measure</scope>
              <count_list>
                <count group_id="O1" value="3"/>
              </count_list>
            </analyzed>
          </analyzed_list>
          <class_list>
            <class>
              <title>Baseline Mean Adult Score for Anxiety</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="46.77" spread="13.33"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-Up Mean Adult Score for Anxiety</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="46.87" spread="6.45"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Adult Score for Depression</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="42.70" spread="10.05"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-Up Mean Adult Score for Depression</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="44.23" spread="9.53"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Adult Score for Fatigue</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="45.23" spread="9.53"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-Up Mean Adult Score for Fatigue</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="42.80" spread="4.60"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Adult Score for Upper Extremity Function</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="48.97" spread="8.37"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-Up Mean Adult Score for Upper Extremity Function</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="53.80" spread="0.00"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Adult Score for Lower Extremity Function</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="58.60" spread="0.00"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-Up Mean Adult Score for Lower Extremity Function</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="58.60" spread="0.00"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Adult Score for Cognitive Function</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="47.03" spread="15.52"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-Up Mean Adult Score for Cognitive Function</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="46.73" spread="5.01"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Adult Score for Emotional and Behavioral Dyscontrol</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="51.10" spread="14.97"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-Up Mean Adult Score for Emotional and Behavioral Dyscontrol</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="41.37" spread="9.98"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Adult Score for Well-being</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="54.30" spread="4.91"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-Up Mean Adult Score for Well-being</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="55.70" spread="6.59"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Adult Score for Sleep Disturbances</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="47.03" spread="7.49"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-Up Mean Adult Score for Sleep Disturbances</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="43.93" spread="4.90"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Adult Score for Participation in Social Activities</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="53.00" spread="7.41"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-Up Mean Adult Score for Participation in Social Activities</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="51.03" spread="8.01"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Adult Score for Satisfaction in Social Activities</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="47.70" spread="3.28"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-Up Mean Adult Score for Satisfaction in Social Activities</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="49.23" spread="2.54"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Baseline Mean Adult Score for Stigma</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="53.90" spread="8.80"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
            <class>
              <title>Follow-Up Mean Adult Score for Stigma</title>
              <category_list>
                <category>
                  <measurement_list>
                    <measurement group_id="O1" value="56.47" spread="6.25"/>
                  </measurement_list>
                </category>
              </category_list>
            </class>
          </class_list>
        </measure>
      </outcome>
    </outcome_list>

    <reported_events>
      <time_frame>6 months</time_frame>
      <desc>Serious adverse events include those events that: result in death; are life-threatening; require hospitalization or prolongation of existing hospitalization; create persistent, significant disability, or birth defects.&#xD;
Expected adverse events are those that are identified in the research protocol as having been previously associated with participation in the study. All reported adverse events will be classified using the Common Terminology Criteria for Adverse Events Version 4.0.</desc>
      <group_list>
        <group group_id="E1">
          <title>Cannabidiol/ Epidiolex</title>
          <description>All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome.&#xD;
Cannabidiol: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.</description>
        </group>
      </group_list>
      <serious_events>
        <category_list>
          <category>
            <title>Total</title>
            <event_list>
              <event>
                <sub_title>Total, all-cause mortality</sub_title>
                <counts group_id="E1" subjects_affected="0" subjects_at_risk="10"/>
              </event>
              <event>
                <sub_title>Total, serious adverse events</sub_title>
                <counts group_id="E1" subjects_affected="0" subjects_at_risk="10"/>
              </event>
            </event_list>
          </category>
        </category_list>
      </serious_events>
      <other_events>
        <frequency_threshold>0</frequency_threshold>
        <default_assessment>Systematic Assessment</default_assessment>
        <category_list>
          <category>
            <title>Total</title>
            <event_list>
              <event>
                <sub_title>Total, other adverse events</sub_title>
                <counts group_id="E1" subjects_affected="7" subjects_at_risk="10"/>
              </event>
            </event_list>
          </category>
          <category>
            <title>Ear and labyrinth disorders</title>
            <event_list>
              <event>
                <sub_title>Dizziness</sub_title>
                <counts group_id="E1" events="3" subjects_affected="2" subjects_at_risk="10"/>
              </event>
            </event_list>
          </category>
          <category>
            <title>Gastrointestinal disorders</title>
            <event_list>
              <event>
                <sub_title>Diarrhea</sub_title>
                <counts group_id="E1" events="2" subjects_affected="2" subjects_at_risk="10"/>
              </event>
              <event>
                <sub_title>Nausea</sub_title>
                <counts group_id="E1" events="3" subjects_affected="2" subjects_at_risk="10"/>
              </event>
            </event_list>
          </category>
          <category>
            <title>Hepatobiliary disorders</title>
            <event_list>
              <event>
                <sub_title>Increased alanine aminotransferase</sub_title>
                <counts group_id="E1" events="2" subjects_affected="2" subjects_at_risk="10"/>
              </event>
              <event>
                <sub_title>Increased aspartate aminotransferase</sub_title>
                <counts group_id="E1" events="2" subjects_affected="2" subjects_at_risk="10"/>
              </event>
            </event_list>
          </category>
          <category>
            <title>Metabolism and nutrition disorders</title>
            <event_list>
              <event>
                <sub_title>Decreased appetite</sub_title>
                <counts group_id="E1" events="3" subjects_affected="1" subjects_at_risk="10"/>
              </event>
              <event>
                <sub_title>Fatigue</sub_title>
                <counts group_id="E1" events="2" subjects_affected="2" subjects_at_risk="10"/>
              </event>
            </event_list>
          </category>
          <category>
            <title>Nervous system disorders</title>
            <event_list>
              <event>
                <sub_title>Headache</sub_title>
                <counts group_id="E1" events="3" subjects_affected="2" subjects_at_risk="10"/>
              </event>
            </event_list>
          </category>
          <category>
            <title>Psychiatric disorders</title>
            <event_list>
              <event>
                <sub_title>Irritable mood</sub_title>
                <counts group_id="E1" events="1" subjects_affected="1" subjects_at_risk="10"/>
              </event>
              <event>
                <sub_title>Jitteriness</sub_title>
                <counts group_id="E1" events="1" subjects_affected="1" subjects_at_risk="10"/>
              </event>
            </event_list>
          </category>
          <category>
            <title>Skin and subcutaneous tissue disorders</title>
            <event_list>
              <event>
                <sub_title>Darkening of port-wine birthmark</sub_title>
                <counts group_id="E1" events="1" subjects_affected="1" subjects_at_risk="10"/>
              </event>
              <event>
                <sub_title>Mouth sores</sub_title>
                <counts group_id="E1" events="1" subjects_affected="1" subjects_at_risk="10"/>
              </event>
            </event_list>
          </category>
        </category_list>
      </other_events>
    </reported_events>

    <certain_agreements>
      <pi_employee>Principal Investigators are NOT employed by the organization sponsoring the study.</pi_employee>
      <restrictive_agreement>There is NOT an agreement between Principal Investigators and the Sponsor (or its agents) that restricts the PI's rights to discuss or publish trial results after the trial is completed. </restrictive_agreement>
    </certain_agreements>
    <limitations_and_caveats>Due to the COVID-19 , visits transitioned from an in-person setting to a remote setting. Four subjects had baseline testing conducted in person and follow-up virtually, and 5 subjects had both baseline and follow-up testing virtually. The differences in testing environment and the context of the pandemic may have affected results. Also, due to variability in subject age and the small number of subjects, some participants were outside of assessment age norms.</limitations_and_caveats>
    <point_of_contact>
      <name_or_title>Dr. Anne Comi</name_or_title>
      <organization>Kenndey Krieger Institute</organization>
      <phone>443-923-9127</phone>
      <email>comi@kennedykrieger.org</email>
    </point_of_contact>
  </clinical_results>
</clinical_study>


download date: ClinicalTrials.gov processed this data on September 20, 2023
link text: Link to the current ClinicalTrials.gov record.
url: https://clinicaltrials.gov/ct2/show/NCT04447846
org study id: IRB00204427
nct id: NCT04447846
lead sponsor: 
collaborator: 
collaborator: 
has dmc: Yes
is fda regulated drug: Yes
is fda regulated device: No
is us export: Yes
The purpose of this study is to better understand the utility of cannabidiol (CBD/ Epidiolex)
      for improving the treatment of cognitive impairments in Sturge-Weber syndrome (SWS).
The investigators hope to gain an understanding of the utility of pharmaceutical grade CBD
      used for the treatment of cognitive impairments in SWS in this open-label study. Anecdotal
      evidence from a phase I trial investigating the use of CBD for medically refractory seizures
      suggests CBD may also have a beneficial effect on cognition, mood, and behavior. The
      investigators hypothesize that CBD/ Epidiolex will improve SWS brain function resulting in
      improved cognitive function, social interactions, mood, motor function and behavior, as well
      as reduced migraines. This is an open-label prospective oral drug trial of Epidiolex in 10
      subjects. Assessments will be done at baseline and repeated after 6 months on study drug.
allocation: N/A
intervention model: Single Group Assignment
primary purpose: Treatment
masking: None (Open Label)
measure: List Sorting Working Memory Test
time frame: Baseline, Follow-up (6 months)
description: Data on cognitive function was collected using the List Sorting Working Memory Test from the NIH Toolbox. Data was collected on working memory performance, which was transformed into a t-score from 0 to 100 where a higher t-score indicates better performance. T-score of 50 indicates the population mean with a standard deviation of 10. Data was collected at baseline and after 6 months on study drug.
measure: Picture Vocabulary Test
time frame: Baseline, Follow-up (6 months)
description: Data on cognitive function was collected using the Picture Vocabulary subtest from the NIH Toolbox. Single words are presented via an audio file, paired simultaneously with 4 screen images of objects, actions, and/or depictions of concepts. The task is to pick the picture that matches the spoken word. Performance on the task was transformed into a t-score from 0 to 100 where a higher t-score indicates better performance. T-score of 50 indicates the population mean with a standard deviation of 10. Data was collected at baseline and after 6 months on study drug.
measure: Seizure Frequency
time frame: Baseline, Follow-up (6 months)
description: Change in seizure frequency by seizure score at pre-treatment baseline and after six months. The seizure score is taken from the Sturge-Weber Neuroscore on a scale of 0 to 4 where 0=none, 1=1+. but controlled, 2=Breakthrough, 3=monthly, 4=Weekly+. Higher scores indicate worse outcome.
measure: Migraine Severity
time frame: Baseline, Follow-up (6 months)
description: Data on migraine severity will be collected using patient responses to questions on a standard six-point scale. Data will be collected on the frequency of an event (e.g. feelings of frustration, performance of daily activities) which will be transformed into a score from 0 to 100 where higher scores indicate a less migraine severity and better outcomes. Data will be collected at baseline and after 6 months on study drug.
measure: Modified House Classification Scores
time frame: Baseline, Follow-up (6 months)
description: Data on motor function was collected using a Modified House Classification. Data was collected on the ability to complete a task with the subject's non-dominant hand which was transformed into a score from 1 to 8 and 0 to 32 where higher scores indicate better motor function. Data was collected at baseline and after 6 months on study drug.
measure: Erhardt Developmental Prehension Assessment Scores
time frame: Baseline, Follow-up (6 months)
description: Data on motor function was collected using the Erhardt Developmental Prehension assessment. Data was collected on the ability to complete a task with each hand, which was scored as age equivalence of task performance (in months). Higher scores indicate better motor function. Data was collected at baseline and after 6 months on study drug.
measure: Pediatric Evaluation of Disability Inventory Computer Adapted Test
time frame: Baseline, Follow-up (6 months)
description: Data on motor function was collected using the Pediatric Evaluation of Disability Inventory Computer Adapted Test. Data was collected on the subject's ability to complete tasks involved in daily activities, mobility, and social/ cognitive activities. The data was transformed into scaled scores ranging from 20 to 80, where higher scores indicate better motor function. Data was collected at baseline and after 6 months on study drug.
measure: ABILHAND Questionnaire
time frame: Baseline, Follow-up (6 months)
description: Data on motor function was collected using the ABILHAND questionnaire, a measure of manual ability for adults with upper limb impairments. Data was collected on the subject's ability to complete daily activities that involve the upper limbs. Score was collected as a patient measure with scores ranging from -10 to 10. Higher scores indicate better motor function. Data was collected at baseline and after 6 months on study drug.
measure: Wechsler Intelligence Scale for Children, Fifth Edition (WISC-V) or Wechsler Adult Intelligence Scale (WAIS-IV)
time frame: Baseline, Follow-up (6 months)
description: Data on cognitive function was collected using selected subtests, from either the Wechsler Intelligence Scale for Children, Fifth Edition (WISC-V) or the Wechsler Adult Intelligence Scale (WAIS-IV). For the WISC-V and WAIS-IV subtests selected, scaled scores were derived from the normative data which account for age and demographic information. The selected WISC-V/WAIS-IV subtests were as follows: Digit Span, Symbol Search, Coding and Processing Speed; the first three subtests were scored from 0-10, the fourth subtest from 0-100, and for all subtests higher score is better. The selected subtests, from the WISC-V and the WAIS-IV, were combined to increase statistical power.
Statistically rare changes in individual test scores were determined using a reliable change index methodology based upon normative information for these assessments.
measure: Pediatric Neurological Quality of Life (Neuro-QoL)
time frame: Baseline, Follow-up (6 months)
description: Data on cognitive function was collected using Neurological Quality of Life scales from the NIH Toolbox. Data on frequency of an event (e.g. forgetting schoolwork) was collected and transformed into a t-score from 0 to 100 where higher t-scores indicate worse anger, worse anxiety, worse pain, better social relationships, worse stigma, worse depression, better cognitive function, and worse fatigue. T-score of 50 indicates the population mean with a standard deviation of 10. Data was collected at baseline and 6 months on study drug.
measure: Behavior Rating Inventory of Executive Function, Second Edition (BRIEF-2)
time frame: Baseline, Follow-up (6 months)
description: Data on executive function was collected using the Behavior Rating Inventory of Executive Function, Second Edition (BRIEF-2). Data on the frequency of an event (e.g. becomes upset too easily) was collected and transformed into a t-score. The following BRIEF-2 subtests were evaluated: Behavioral Regulation Index, Emotional Regulation Index, Cognitive Regulation Index, and Global Executive Composite. For each BRIEF-2 subtest, t-scores range from 0 to 100; a score of 50 indicates the population mean with a standard deviation of 10. For all BASC-3 subtests, higher scores are worse outcome.
measure: Social Responsiveness Scale, Second Edition (SRS-2)
time frame: Baseline, Follow-up (6 months)
description: Data on social function was collected using the Social Responsiveness Scale-Second Edition (SRS-2). Data on a child's ability to engage in emotionally appropriate reciprocal social interactions in naturalistic settings was collected and transformed into a t-score from 0 to 100 where higher scores indicate greater impairment in social function. T-score of 50 indicates the population mean with a standard deviation of 10. Data was collected at baseline and 6 months on study drug.
measure: Behavioral Assessment System for Children, Third Edition (BASC-3)
time frame: Baseline, Follow-up (6 months)
description: Data on behavioral function was collected using the Behavioral Assessment System for Children, Third Edition (BASC-3). Data on the frequency of a behavior (e.g. avoids eye contact) was collected and transformed into a t-score. Subscales for the BASC-3 scored were: External Problems Composite, the Internal Problems Composite, the Behavioral Symptoms Index, and the Adaptive Skills Composite. For the first three subscales, lower t-score indicates better outcome; for the fourth, a lower t-score indicates worse outcome. T-scores for all the BASC-3 subscales range from 0 to 100, and a t-score of 50 indicates the population mean with a standard deviation of 10.
measure: Screen for Child Anxiety Related Disorders (SCARED)
time frame: Baseline, Follow-up (6 months)
description: Data on anxiety was collected using the Screen for Child Anxiety Related Disorders (SCARED). Data on the truthfulness of a statement (e.g. I am nervous) was collected and transformed into a score from 0 to 82 where higher scores indicate greater anxiety. Data was collected at baseline and 6 months on study drug.
measure: Quality of Life in Childhood Epilepsy Questionnaire (QOLCE-55)
time frame: Baseline, Follow-up (6 months)
description: Data on quality of life was collected using the Quality of Life in Childhood Epilepsy Questionnaire (QOLCE-55). Data on the frequency of an event (e.g. had trouble concentrating on a task) was collected and transformed into a score from 0 to 100 where higher scores reflect better quality of life. Data was collected at baseline and 6 months on study drug.
measure: Safety of Epidiolex
time frame: Baseline, Follow-up (6 months)
description: Safety of Epidiolex was measured by the number of adverse events and serious adverse events that result from study drug.
measure: Neuroscore
time frame: Baseline, Follow-up (6 months)
description: Data on neurological function was collected using the Neuroscore. Data on frequency of seizures, extent of hemiparesis, assessment of visual field cut, and degree of cognitive functioning was transformed into a score from 0 to 15 where higher scores indicate worse neurologic function. Data was collected at baseline and 6 months on study drug.
measure: Port-wine Birthmark Score
time frame: Baseline, Follow-up (6 months)
description: Data on facial port-wine birthmarks was collected using the Port-wine Birthmark Score. Data on percent of face covered, thickness of birthmark, and darkness of birthmark color was collected and transformed into a score from 0 to 43 where higher scores indicate greater severity and greater surface area involved. Data was collected at baseline and 6 months on study drug.
measure: Adult Neurological Quality of Life (Neuro-QoL)
time frame: Baseline, Follow-up (6 months)
description: Data on cognitive function was collected using Neurological Quality of Life scales from the NIH Toolbox. Data on frequency of an event was collected and transformed into a t-score from 0 to 100. Higher t-scores indicate better communication, better ability to participate in social activity, worse anxiety, worse depression, worse emotional and behavioral dyscontrol, worse fatigue, better positive affect, worse sleep disturbance, better lower and upper extremity functions, worse stigma, better satisfaction with social roles, and better cognitive function. T-score of 50 indicates the population mean with a standard deviation of 10. Data was collected at baseline and 6 months on study drug.
arm group label: Cannabidiol/ Epidiolex
arm group type: Experimental
description: All subjects will receive the experimental Epidiolex (cannabidiol) oral solution to be taken at home twice a day, and will be treated on an outpatient basis. The drug will be taken for 24 weeks unless the subject chooses to participate in the extension phase of the study, in which case the subject will continue to receive the drug for one additional year or until the drug is approved for clinical use for the treatment of cognitive impairments in patients with Sturge-Weber syndrome.
intervention type: Drug
intervention name: Cannabidiol
description: Initiation of treatment will begin with 5 mg/kg/day given in two divided doses. The dose will be increased by 5 mg/kg/day after seven days and then by 5 mg/kg/day every seven days up to a maximum dose of 20 mg/kg/day given.
arm group label: Cannabidiol/ Epidiolex
other name: Epidiolex
other name: CBD
criteria: 
gender: All
minimum age: 3 Years
maximum age: 50 Years
healthy volunteers: No
last name: Anne M Comi, MD
role: Principal Investigator
affiliation: Hugo W. Moser Research Institute at Kennedy Krieger, Inc.
facility: 
country: United States
url: https://www.kennedykrieger.org/patient-care/centers-and-programs/sturge-weber-syndrome-center
description: Hunter Nelson Sturge-Weber Center Website
responsible party type: Sponsor-Investigator
investigator affiliation: Hugo W. Moser Research Institute at Kennedy Krieger, Inc.
investigator full name: Anne Comi, MD
investigator title: Principal Investigator, Director Sturge-Weber Center, Kennedy Krieger Institute, Professor Johns Hopkins University School of Medicine
mesh term: Sturge-Weber Syndrome
mesh term: Brain Stem Infarctions
mesh term: Klippel-Trenaunay-Weber Syndrome
mesh term: Syndrome
mesh term: Cannabidiol
provided document: 
participant flow: 
baseline: 
outcome list: 
reported events: 
certain agreements: 
limitations and caveats: Due to the COVID-19 , visits transitioned from an in-person setting to a remote setting. Four subjects had baseline testing conducted in person and follow-up virtually, and 5 subjects had both baseline and follow-up testing virtually. The differences in testing environment and the context of the pandemic may have affected results. Also, due to variability in subject age and the small number of subjects, some participants were outside of assessment age norms.
point of contact: 