In March 2007, a 47-year-old woman with a history of bilateral optic neuritis at age 35 experienced her first seizure. One month later, she experienced a second seizure and was started on lamotrigine. She presented to our institution as an elective admission to the EMU 6 months after seizure onset. Many right temporal seizures (clinical semiology of paresthesias over left face and arm) were observed and the interictal EEG was notable for right periodic lateralized discharges (PLDs; Figure ). MRI showed abnormal T2 signal in the right frontal cortex, right thalamus as well as a small area of contrast enhancement in the right temporal uncus. MR SPECT showed normal choline:creatinine ratio. Serum studies were notable for the following normal/negative studies: ANA, ACE, anti-TPO, Lyme, paraneoplastic antibody (ANNA-1, ANNA-2, ANNA-3, AGNA-1, PCA-1, PCA-2, PCA-Tr, CRMP-5, VGKC). CSF evaluation was significant for a mild lymphocytic pleocytosis, negative HSV and CMV PCR, and cytology negative for malignant cells. Ultimately, after trials of several AEDs including lamotrigine, levetiracetam, and carbamazepine, oxcarbazepine and phenytoin were utilized for seizure control.\nOver the next 3 years, the patient had several admissions to our institution for breakthrough seizures. Her seizure semiologies over this time frame were isolated left face and arm paresthesias, focal motor seizures involving left face and arm as well as secondarily generalized tonic/clonic seizures. A static mild left hemiparesis, most affecting the left hand, was also noted during this time. Further diagnostic work-up over one of these later admissions was notable for normal CSF cell counts, a CT scan of the chest, abdomen, and pelvis negative for malignancy, and repeat negative paraneoplastic panel evaluation. At this point, the patient was lost to follow-up.\nShe re-presented to our institution 5 years later after she experienced a generalized convulsion. Collateral history indicated that she had become unable to live at home unassisted and often had confusional and agitated episodes. Since last seen at our institution, her AED regimen had been changed to levetiracetam and valproic acid. In addition, a third lumbar puncture was performed and notably NMDA-R antibody testing was negative. The patient was found to be in non-convulsive status epilepticus and treated with benzodiazepines and augmented doses of her AEDs. CT imaging done on this admission revealed marked right hemisphere atrophy. Continuous EEG monitoring showed both right frontal and temporal spikes and left temporal slowing. Clinical exam was significant for perseverative speech, inability to follow complex commands and a moderate left hemiparesis. Given the patient’s marked hemiatrophy, refractory epilepsy, and static neurologic deficits, she was diagnosed with RE and monthly IVIG was initiated.\nMR imaging performed showed marked right hemisphere volume loss, affecting both gray and white mater (Figure ). Despite the marked asymmetry on imaging findings, EEG monitoring during this advanced stage of disease revealed bihemispheric electrographic pathology; in addition to PLDs in the right posterior quadrant, PLDs and spikes were observed over the left temporal region as well as bilateral independent frontal spikes (Figure ). By family and caregiver report, patient’s overall seizure frequency decreased over the 5-month period she was treated with immunotherapy. As there was no significant change in the patient’s overall neurologic functional status, decision was made by family to discontinue IVIG infusions and pursue hospice.\nWritten informed consent was obtained from patients or next of kin for publication of these three case reports.
A 5 year old male child presented with complaints of multiple episodes of clonic seizures of the left upper and lower limb since the last 8 months, each episode was lasting for 5–15 min with a prolonged postictal state. He was tre ated with anticonvulsant drugs. He had no history of blood transfusions, neurosurgery, traumatic head injury, proven allergies, and family history of epilepsy. Antenatal, perinatal and postnatal history was normal. The seizures were progressively increasing in frequency and severity since last month. The episodes also involved a sudden loss of consciousness and bladder incontinence. Because of that, he admitted to our neurology department. On examination, the child was disoriented. His vitals were stable. Central nervous system examination showed left-sided hemiparesis with exaggerated tendon reflexes on the left side. Muscle power Medical Research Council (MRC) was grade 4 on the left upper and lower limb, but a full 5 on the right upper and lower limbs. There were no signs of meningeal irritation, and cranial nerves were intact. Cerebrospinal fluid analysis was normal, (PCR) for Herpes Simplex Virus, EBV, measles and CMV, appeared to be negative.\nThe patient had a progression to epilepsia partialis continua within 9 months after first focal seizure and impairment in cortical functioning.\nThe MRI Flair showed high signal intensity area in right temporal lobe ().\nMRI T1 and T2 showed atrophy in right partial and temporal lobes ().\nEEG showed slow activity with focal right frontal polyphasic spikes paroxysmal discharges ().\nOn arrival he was give diazepam and phenytoin infusion. Later treatment was started on oral levetiracetam, clonazepam, lamotrigin, Carbamzepine and valproate sodium.\nAfter 25 days of treatment, patient continued to develop seizures and her right-sided weakness persisted, progressed and became worsen. During these episodes of fits, child was started on intravenous steroids.\nTo slow hemispheric atrophy, anti-inflammatory treatment with tacrolimus was given.\nFollow up MRI revealed effacement of cortical sulci and also asymmetry between two cerebral hemisphere with atrophy of right cerebral hemisphere.\nThe patient was treated with regular dosing of IVIG. A dose interval of 4 weeks was determined. Despite regular IVIG dosing for approximately 6 months as well as concomitant AED therapy, the patient's epilepsy remained refractory. Functional Hemispherectomy was performed by the complete removal of the cortex and white matter, sparing the basal ganglia. After surgery, the child had mild episodes of seizures. Unfortunately, he died because of meningitis.