A 25-month-old male with a history of mild reactive airway disease and sickle cell trait was noted by his mother to be breathing slowly and erratically 2 h after being laid down for a nap. Emergency medical services were called and paramedics encountered a pulseless, apneic patient. Cardiopulmonary resuscitation was immediately initiated. Return of spontaneous circulation was achieved in the ambulance on route to our institution’s emergency department.\nOn arrival, physical examination revealed an afebrile toddler, unresponsive to voice or noxious stimulation with agonal respirations, a blood oxygen saturation of 93% with bag-mask-assisted ventilation, and a blood pressure of 99/65 mmHg (mean arterial pressure 76 mmHg). Neurologic examination demonstrated 3 mm, non-reactive pupils and global hyperreflexia. Orotracheal intubation was performed emergently. Initial laboratory evaluation revealed normal electrolytes (Na+ 138 mEq/L, K+ 4.9 mEq/L, Cl− 104 mEq/L, Ionized Ca2+ 5.2 mEq/L), leukocyte count (11,100/mm3), venous blood gases (pH 7.37, PvCO2 44, PvO2 47, 25.7), and serum lactate (0.9 mmol/L). Upon further interrogation, the patient’s mother reported the presence of prescription opiates (extended release morphine) in the household, which she uses for pain control secondary to frequent sickle cell anemia crises. A total of two doses of naloxone (0.1 mg/kg i.v. push each) were administered, after which the patient regained pupillary reactivity and began moving all four extremities spontaneously. A urine toxicology panel was obtained, which was positive for morphine.\nA non-contrast head CT was obtained (Figure B), which revealed acute hydrocephalus and extensive, symmetric bilateral cerebellar hemispheric hypoattenuation consistent with severe cerebellar edema that resulted in compression of the fourth ventricle and displacement of the midbrain and pons superiorly. Cervicomedullary junction compression secondary to cerebellar tonsillar herniation was also noted. Neurosurgery was consulted and the patient was emergently taken to the operating room for the 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 brain MRI obtained on postoperative day 1 demonstrated cerebellar and cervicomedullary junction decompression, with extensive restricted diffusion in the remaining cerebellar parenchyma (Figure B). Multifocal areas of diffusion restriction were also noted bilaterally in the anterior cerebral artery-middle cerebral artery and middle cerebral artery-posterior cerebral artery watershed territories involving portions of the frontal, occipital, and temporal lobe white matter. An MRA/MR venogram demonstrated no craniocervical or intracranial arterial abnormalities or evidence of cortical or dural venous sinus thrombosis. The patient was extubated on postoperative day 5. Assessment at postoperative day 18 demonstrated an easily arousable child, with spontaneous eye opening, equally round and reactive pupils, and with purposeful movements of all four extremities.\nOutpatient follow-up at 29 months of age (3 months after presentation) demonstrated an active child speaking in full sentences. He was able to ambulate independently, albeit with a slightly widened base of support, and mild left lower extremity spasticity which did not limit his ability to climb or descend stairs with rail support.
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).