Category: Clinical Sciences/Health Conditions
Case Diagnosis: Acute Disseminating Encephalomyelitis (ADEM)
Case Description: A 23-year-old female with history of prediabetes and morbid obesity presented with bilateral blindness following 10 days of headache with viral prodrome. Initial ocular ultrasound was negative for retinal detachment; head CT was negative for a cerebrovascular event. Pseudotumor cerebri was suspected but lumbar puncture attempt failed, prompting EVD placement. Subsequent MRI revealed multiple demyelinating lesions in periventricular deep white matter of the basal ganglia, midbrain, cerebellum, and bilateral optic nerves. EVD was discontinued after ruling out pseudotumor cerebri, and further lab work returned negative for meningitis, thus increasing suspicion for Acute Disseminating Encephalomyelitis (ADEM). High-dose steroid therapy and plasma exchange were initiated without success. Rituxan and methotrexate were trialed next for refractory symptoms. She ultimately presented to inpatient rehabilitation with persistent bilateral vision loss, balance deficits, and minor cognitive deficits.
Peak incidence of ADEM follows a bimodal trend in pediatric and elderly populations, with scant literature of ADEM manifesting in age groups lying in between. ADEM may present as a combination of CNS symptoms, including aphasia, ataxia, optic neuritis, and seizures. Work-up begins with CT and LP and progresses to MRI when inconclusive. Findings should be distinguished from those of multiple sclerosis (MS), the primary differential diagnosis. Typical treatment course includes high dose IV glucocorticoids with refractory cases then progressing through IVIG, plasma exchange, and cyclophosphamide or other immunosuppressants.
ADEM presented atypically in this young adult as a 10-day course of headache followed by bilateral blindness. While treatment courses are similar, disease course varies, making it important to keep ADEM on the differential, even in the setting of an atypical presentation, when evaluating for multiple sclerosis. Rehabilitation teams taking care of these patients should be aware of adaptive technologies available to them and how to effectively transition these patients back into the community upon discharge.
Megha Mandalaywala– Resident Physician, Carolinas Rehabilitation
Alexander Brahmsteadt– MS IV, Arizona College of Osteopathic Medicine
Eric Westerbeck– Resident Physician, Carolinas Rehabilitation
Kelly Crawford– Clinical Director of Brain Injury Medicine at Carolinas Rehabilitation, Carolinas Rehabilitation