Clinical Reasoning: A patient with a history of encephalomyelitis and recurrent optic neuritis

A 4-year-old girl presented to an outside hospital after waking up with inability to stand or walk. She had a viral prodrome with fever for several days. Brain and spine MRIs revealed lesions in the left caudate, bilateral insular cortex, right thalamus, and right temporal cortex as well as longitudinally extensive cervical and thoracic cord lesions. Spinal fluid showed pleocytosis with 200 white blood cells (mixed neutrophils and lymphocytes), elevated CSF protein (103 mg/dL), and negative bacterial and viral cultures and PCR. Oligoclonal bands were negative. During the hospitalization, she developed encephalopathy and was diagnosed with acute disseminated encephalomyelitis (ADEM). She received a course of IV steroids followed by an oral steroid taper, and her neurologic symptoms resolved fully. However, within 2 weeks, she experienced painless bilateral vision loss. A repeat brain and orbit MRI revealed swelling in the bilateral optic nerves and decrease in size or resolution of prior brain lesions. Optic neuritis (ON) was diagnosed based on clinical and radiographic presentation. She was given a course of IV immunoglobulin, followed by IV steroids, and a longer steroid taper.
Source: Neurology - Category: Neurology Authors: Tags: All Pediatric, Optic neuritis; see Neuro-ophthalmology/Optic Nerve, Acute disseminated encephalomyelitis, Transverse myelitis RESIDENT AND FELLOW SECTION Source Type: research