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.
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 ().