A 29-year-old man was referred to the emergency department (ED) due to severe headache and behavioral changes.\nThe symptoms had started 12 days before admission with the headache considered as migraine and treated by oral pain killers. The patient came back to the ED 2 days later with persisting headache and right-sided paresthesia. Brain computed tomography (CT) failed to reveal any abnormality, and the patient was sent home again with nonsteroidal anti-inflammatory drugs and tramadol.\nTwo days later, he developed severe behavioral and cognitive changes with incomprehensible vocalizations, anterograde amnesia, aggressiveness towards his family members, and agitation. Sleeping rhythm was inverted with alternating periods of apathy/somnolence and agitation.\nPatient's medical history was unremarkable, and he did not take any medication. Occasional use of tobacco and alcohol was reported. We learned from his father that he had started regular cocaine consumption 3 months ago but should have stopped about one week before admission.\nOn admission, the patient was awake, but disorientated in time and place, and did not properly answer to questions. There was no sign of lateralization and cerebellar tests were normal. Neck stiffness and photophobia were noted.\nRoutine laboratory investigations were unremarkable, as well as blood and urine toxicological screening. Repeated brain CT was also normal. Lumbar puncture showed a normal opening pressure, and cerebrospinal fluid (CSF) analysis revealed 7 mononuclear cells/mm3 and a high protein level at 224 mg/dL. The albumin quotient was highly increased (37.9, normal value <6.5 under 40 years of age), confirming, together with the further brain magnetic resonance imaging (MRI) findings, significant alteration of the blood-CSF barrier permeability. Microbiological and viral CSF tests were negative. No CSF-specific IgG oligoclonal bands were found. As infectious encephalitis was ruled out, antimicrobial and antiviral therapy was interrupted.\nWhile staying in the ICU, the patient had been less agitated but remained disoriented in time and space and had suffered from transcortical sensitive aphasia. Brain MRI was performed 14 days after symptom onset which showed ubiquitous small lesions within white matter displaying high signal intensity on diffusion-weighted images and water diffusivity restriction on apparent diffusion coefficient (ADC) maps. The radiologist initially considered the hypothesis of disseminated ischemic foci due to cocaine-related vasoconstriction (not illustrated). Brain MRI examination was repeated after 3 days and showed numerous additional punctate lesions involving the corpus callosum, together with abnormal meningeal enhancement and contrast agent leakage in the CSF (Fig. ).\nRetinal fluoroangiography revealed multiple segmental occlusions of the central retinal artery (Fig. ), and an audiogram showed bilateral but asymmetric sensorineural hearing loss, with a left predominance.\nAfter the diagnosis of SS, bolus corticosteroid therapy was initiated using methylprednisolone 1 g daily for 5 days, followed by tapering oral doses. Toxicological investigations were completed by hair analysis using ultra-performance liquid chromatography tandem mass spectrometry operating in positive electrospray mode (Quattro Premier, Waters, Milford, MA, USA). Hair samples were cut into six segments of 1 cm, and the presence of cocaine, levamisole, opioids, and several amphetamines was investigated. Only cocaine together with metabolites, levamisole, and tramadol were detected. Quantification was possible using deuterated analogues: cocaine-D3 for cocaine and norcocaine, benzoylecgonine-D8 for benzoylecgonine, methylecgonine-D3 for methylecgonine, 6-acetylmorphine-D6 for levamisole, and tramadol-D3 for tramadol. The following concentration ranges were found in the hair: cocaine (>5,000 pg/mg), benzoylecgonine (>5,000 pg/mg), methylecgonine (68–113 pg/mg), norcocaine (581 to >1,000 pg/mg), levamisole (73–139 pg/mg), and tramadol (1,378–2,155 pg/mg). The patient was transferred from our institution to a rehabilitation center 13 days after admission. Cognitive status progressively improved, but the patient remained dysexecutive and had features of frontal lobe syndrome. Five weeks after discharge, the patient presented with a subacute left-eye central scotoma and altitudinal visual field deficit while still on a daily oral dose of 16 mg methylprednisolone. New retinal branch artery occlusions were documented on fluoroangiography (Fig. ). However, brain MRI did not show additional microvascular ischemic lesions when compared to previous examination, and lepto-meningeal enhancement had significantly decreased (not shown). This relapse (or progression of the initial lesions) was again treated by repeated methylprednisolone pulse therapy followed by oral tapering. Immunosuppressive treatment with mycophenolate mofetil was started. At 10-month follow-up, the patient was asymptomatic. He resumed work and did not report any residual cognitive deficit, as confirmed by neuropsychological testing performed 3 months after the initial episode.
A previously healthy 10-month-old female was found by her mother after waking in the morning to have slow, labored breathing. She was unresponsive to voice and tactile stimulation. This prompted a call to 911. On arrival, emergency medical services personnel noted that the infant was non-arousable, bradypneic, and stridorous, with a blood oxygen saturation of 80% on room air. She was transported to the emergency department of a regional hospital, where orotracheal intubation was immediately performed. Her blood pressure on arrival was 93/53 mmHg (mean arterial pressure 66 mmHg). Neurologic examination revealed 1 mm hyporeactive pupils bilaterally, global hypertonia, and hyperreflexia. A non-contrast computed tomography (CT) scan of the head was obtained. Arterial blood gas demonstrated a combined metabolic and respiratory acidosis (pH 7.15, PaCO2 29, PaO2 103, 10). Additional laboratory evaluation revealed mild transaminitis (aspartate aminotransferase 100 IU/L, normal = 8–50 IU/L; alanine aminotransferase 55 IU/L, normal = 7–45 IU/L) and leukocytosis (31,000/mm3, normal = 6,000–11,000/mm3). A broad differential diagnosis of respiratory failure of unknown origin was proposed, and treatment was pursued for possible allergic reaction or meningitis with two doses of epinephrine (0.085 mg), methylprednisolone (2 mg/kg), diphenhydramine (2 mg/kg), and a meningitic dose of ceftriaxone. The patient was then transferred to our tertiary care center.\nOn admission, workup was expanded with a full toxicology panel, which was positive for oxycodone and its metabolite, oxymorphone. Of note, elevated transaminases raised concern for acetaminophen toxicity; however this was ruled out by a negative result on the toxicology panel. A diagnosis of respiratory failure secondary to opiate ingestion and overdose was established. A total of four doses of naloxone (0.1 mg/kg i.v. push each) were administered with improvement in level of consciousness and reduction of ventilator support that allowed extubation. Continuous naloxone infusion was administered for 5 h thereafter. Results of initial neuroimaging were reviewed and revealed extensive symmetric bilateral cerebellar hypoattenuation (Figure A). The patient was admitted to the pediatric intensive care unit and was placed under close observation with frequent neurological exams.\nOn the third day of hospitalization, she became obtunded, with hyporeactive pupillary reflexes, hypertonia, hypertension, and ocular bobbing. Emergent magnetic resonance imaging (MRI) of the brain was obtained. The MRI demonstrated a pattern of symmetric restricted diffusion in the cerebellar hemispheres (Figure A), right parietal lobule, the heads of the caudate nuclei bilaterally and the right putamen. Additionally, hydrocephalus, elevation of the tectum, cerebellar tonsillar herniation, and an area of increased susceptibility weighted imaging signal in the left perirolandic area were present which suggested hemorrhagic transformation of an infarct.\nThe patient was taken emergently to the operating room for placement of an external ventricular drain, suboccipital craniectomy, and a C1 laminectomy. A significant portion of the edematous and ischemic cerebellar hemispheres and tonsils were resected bilaterally, thereby decompressing the posterior fossa and cervicomedullary junction.\nA postoperative magnetic resonance angiography (MRA) of the head and neck showed normal anterior and posterior circulations. A full coagulopathy workup and echocardiography were unremarkable. No other visceral or musculoskeletal injuries were noted, and an investigation by the child abuse team provided no evidence for physical abuse. After an uneventful postoperative course, the patient was discharged on postoperative day 11.\nFollow-up at 33 months of age (23 months after presentation) demonstrated an active, engaged child who spoke in full sentences. She was able to ambulate, jump, and climb stairs with alternating feet but had mild spasticity and internal rotation of her left leg with gait and occasional dystonic posturing of the left upper extremity.