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 41-years-old-man addicted to chronic cocaine and alcohol abuse was admitted to our emergency department in a stuporous state and with urinary incontinence. In the previous 15 days he had developed dysarthria, mutism with a prominent drowsiness and psychomotor disturbances. A brain MRI, abdomen and chest computed tomography (CT) were performed in another hospital. MRI brain scan showed multiple roundish lesions in the periventricular cerebral white matter.\nOn admission, laboratory tests were within normal limits, and the virological screening together with the Quantiferon, HIV and Treponemal tests was negative. Only C-reactive protein value resulted higher than normal (1.18 mg/L).\nA cerebrospinal fluid (CSF) examination revealed a high albumin level, no oligoclonal bands and a normal immunoglobulin G index. The electroencephalogram (EEG) recorded low α and θ waves.\nUrine toxicologic screening test detected low level of cocaine and it was negative for benzodiazepines, barbiturates, oxycodone, amphetamine, methadone and cannabinoids. The patient’s urine samples were not tested for levamisole. No myelin-oligodendrocyte-glycoprotein (MOG) or aquaporin antibodies 4 were documented.\nFirst, the patient underwent a brain CT study which detected multiple hypointense circular lesions in the bihemispheric white matter surrounded by edema.\nBrain MRI revealed multiple well-circumscribed concentric lamellar lesions in the bihemispheric white matter involving the corpus callosum,; these lesions showed alternate bands of iso-/hypointensity on T1-weighted images and alternate hypo-/hyperintensity on T2-weighted/fluid attenuation inversion recovery (FLAIR) images and presented with a rim of restricted diffusion on diffusion-weighted imaging (DWI; b = 1000), more obvious on the edge of the lesions. Apparent diffusion coefficient (ADC) map confirms alternate bands of diffusion restriction, displaying low signal intensity.\nAfter gadolinium intravenous administration, all the lesions demonstrated an “onion-like” patchy enhancement on post-contrast T1-weighted images.\nDouble inversion recovery (DIR) sequences showed more detailed lamellar structure of the lesions than FLAIR images, thanks to high sensitivity in juxta and subcortical lesions detection.\nMR spectroscopy revealed elevated choline and lactate peaks and decreased N-acetylaspartate peak ().\nMRI of the spine showed no evidence of demyelinating lesions. Stereotactic biopsy obtained from frontal right paraventricular lesion showed gliosis and many swollen reactive eosinophilic astrocytes with perivascular cuffing. Axonal stain showed long stretches of intact axon. In addition, perivascular and periventricular macrophages were found (). These pathologic findings supported a possible inflammatory/immune-mediated etiology.\nThe patient was treated with high-dose oral methylprednisolone for 15 consecutive days, after which his clinical symptoms partially improved.