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.
A 57-year-old man with substance abuse disorder was brought by his family to the emergency room because of strange behavior. Five days prior to admission, he fell off his bicycle and came home with a scalp laceration on the left side of the head. Since then, his family noticed him acting oddly. He was seen walking back and forth in the house between the front door and the back door. He emptied the ice tray and he poured sugar into a shopping bag for no reason. He could no longer perform habitual tasks, such as cooking. His family described his gait as slow “like a zombie.” It was also unusual for him not to leave the house 5 days in a row. He does not see a primary care physician. According to his family, he smoked tobacco regularly (2 packs/day for 40 years), consumed alcohol rarely, and abused marijuana and cocaine.\nThe patient did not complain of headache, nausea, dizziness, and so on; he thinks nothing is wrong with him. Blood pressure was 187/104 mm Hg, heart rate 77 beats/min, respiratory rate 16 breaths/min, temperature 36.6°C, and blood oxygen saturation 100%. Criteria for hypertensive emergency or urgency were not met. He was awake and alert with normal thought content and intact ability to answer questions, but his affect was flat, he was indifferent to his condition, and he exhibited psychomotor retardation. On neurological examination, he was oriented to self, place, and immediate situation, but was oblivious of the month, day, and year. His ability to concentrate was mildly impaired and he pauses frequently when speaking; nevertheless, speech and language function were intact. Cranial nerve functions and motor strength were all normal. Deep tendon reflexes were brisk (3+) in all extremities. Ankle clonus and Babinski sign were absent. Cerebellar signs were also absent. Head computed tomography showed mild diffuse brain atrophy. Blood counts and chemistry were all normal except for mild hyperammonemia (39 µmol/L).\nHe was admitted to the neurology service. Urine was positive for cocaine and benzodiazepine, but only cocaine was found significant on quantitative analysis (levamisole was not tested). Additional studies, including thyroid stimulating hormone; folate; B12; HgbA1C; HIV; hepatitis panel; heavy metal screen for lead, arsenic, and mercury; and blood toxicology for ethanol, methanol, salicylates, acetaminophen, and so on, were all negative. Brain MRI revealed diffuse confluent subcortical white matter hyperintensities on T2-FLAIR and diffusion-weighted imaging (DWI) with low-apparent diffusion coefficient (ADC) in some areas (restricted diffusion) and high ADC in other areas (T2 shine-through effect). The cerebral cortex, cerebellum, basal ganglia, thalamus, and brainstem were all normal (). EEG showed mild diffuse slowing with inordinate amounts of theta waves intermixed with low-voltage beta and alpha activity (). Lumbar puncture was considered but not pursued because we were convinced that he has cocaine-induced TL and we could not justify exposing him to the risk of brain herniation. Albeit infrequent, brain herniation may occur with diffuse brain edema (eg, liver failure).\nImmunosuppressive therapy in cocaine-induced TL has been associated with a favorable or unfavorable outcome (see “Introduction”). Notwithstanding this uncertainty, we chose to treat the patient with a 5-day course of prednisone because of the extent of his disease. Prednisone 60-mg qd was administered on hospital day 4, tapered to 20 mg qd over 5 days, and stopped on hospital day 9. Nutritional therapy and assistance with activities of daily living resulted in gradual normalization of mental status and behavior. After 3 weeks in acute care, the patient was discharged to a rehabilitation facility. He missed his appointments and was seen again in clinic after 5 months. His family pointed out that his cognitive function returned to normal 2 weeks after he was discharged from acute care and at that point he started engaging again in the activities of daily living. During clinic visit, he reported (and his family agreed) that he no longer takes cocaine and other drugs of abuse. Neurological examination and cognitive evaluation did not detect any abnormalities. Follow-up brain MRI showed complete absence of diffusion restriction and near-100% resolution of white matter hyperintensity (). Follow-up EEG showed normal alpha rhythm posteriorly and normal beta activity anteriorly during wakefulness ().