A 25-year-old man with history of cocaine use presented to the emergency room with left-sided upper and lower limb weakness as well as sensory impairment involving the face. Symptoms evolved such that he was bed-ridden after 48 h. He had no history of previous neurological or systemic symptoms. Computed tomography (CT) of the brain showed bilateral periventricular hypodensities. CT angiography of cervical and intracranial vessels was unremarkable. An MRI study of the brain demonstrated increased FLAIR signal deep in the right and left hemispheres (Fig. ), which also displayed patchy gadolinium enhancement (Fig. ) and restricted diffusion (Fig. –). Five days after admission he developed swallowing difficulty, dysarthria, and confusion. In addition to the previously noted MRI abnormalities, repeat imaging showed new lesions in the left internal and external capsules, putamen and corona radiata, right medial pons and left cerebellar peduncle, with heterogenous gadolinium enhancement (Fig. –). The neuroradiological impression was that the lesions were most compatible with demyelinating disease. A urine screen was positive for cocaine, cannabinoids, and oxycodone, and negative for amphetamine, methadone, benzodiazepines, and barbiturates. Other investigations included the following normal or negative results: complete blood count, serum electrolytes, urea, creatinine, glucose, erythrocyte sedimentation rate, antinuclear antibodies, rheumatoid factor, perinuclear and cytoplasmic anti-neutrophil cytoplasmic antibodies (pANCA and cANCA respectively), extractable nuclear antigens, lupus inhibitor, C3, C4, CH50, urinalysis, vitamin B12, angiotensin converting enzyme level, thyroid stimulating hormone level, free T3 and T4, cortisol level, lipid profile, very long chain fatty acids, viral serology for HIV, CMV, Hepatitis A, B, and C, Lyme serology, syphilis screen, blood screen for salicylate, acetaminophen, and ethanol. Liver enzymes (AST, ALT and GGT) were mild to moderately elevated and subsequently normalized over a period of six months. Cerebrospinal fluid (CSF) results included: slightly elevated protein at 0.50 g/l (normal 0.20–0.40 g/l), normal glucose and chloride, normal total cell count (3 × 106/l), negative oligoclonal bands, normal IgG index, elevated albumin index at 9.0 (2.7–4.7), negative cryptoccocal antigen, negative cultures for bacteria and fungi, negative polymerase chain reaction (PCR) studies for CMV and HSV 1–2, and normal cytology.\nHe was treated with intravenous methylprednisolone, 1000 mg daily for five consecutive days, and subsequently underwent plasma exchange. During plasma exchange his neurological condition stabilized and he subsequently achieved good, albeit partial, recovery. At three months follow-up he had mild residual left-sided hemiplegia but was otherwise independent in activities of daily living and starting to return to work. Follow up MRI five months after presentation showed reduction in the size of lesions, no enhancement, and no new lesions (Fig. –). MRI 21 months after the initial presentation also showed no new lesions (not shown).
The patient was a 9-month-old male with a past medical history significant for in utero drug exposure and mild oral aversion, who had otherwise been reportedly well. The day prior to his initial presentation, he had been swimming with his family and was put down for a nap at 1600. Family checked on him at 2100 hours due to not waking up for dinner but found him sleeping and attributed his increased fatigue to his increased activity throughout the day. At 0700 hours the next day, family was still unable to wake the child. He was taken to the nearest emergency department, where his initial vitals were significant for hypoxemia, with oxygen saturation of 77% on room air and mild hypothermia with axillary temperature of 36.2°C. His physical exam was notable for lethargy and decreased level of arousal without external physical exam findings concerning for apparent injury or infection. Labs were significant for hypoglycemia (30 mg/dL), but his mental status did not improve with glucose supplementation. A head CT was performed, and he was found to have bilateral cerebellar hemisphere hypoattenuation with acute swelling on computed tomography (). The presumed diagnosis at the time was acute stroke of unknown etiology, with the possibility of prolonged hypoglycemia and subsequent hypoxia contributing. He was flown to the closest pediatric tertiary care center and admitted to the Pediatric Intensive Care Unit (PICU) for close neurological monitoring and further evaluation.