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).
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.