A-163 Neuropsychological Profile of Early Onset ADEM and AMSAN

Abstract

Abstract Objective: We present the neuropsychological profile of a 9-year-old girl with a history of waxing and waning ataxia, initially diagnosed at 18 months of age, with Acute Disseminated Encephalomyelitis (ADEM), later thought to be axonal motor sensory neuropathy (AMSAN). ADEM is a rapid onset neuroinflammatory disease typically seen in children ages 3-10 following infection, which results in demyelinating lesions. AMSAN is a rare variant of Guillain-Barre´ syndrome causing muscle/sensation difficulty. Common long-term cognitive correlates of ADEM include attention, processing speed and internalizing problems in the context of average overall cognition. Method: Comprehensive neuropsychological evaluation referral by neurology to assist with continued workup given atypical history and unclear academic difficulties. Initial symptom workup revealed signal changes in spinal cord and demyelinating lesions in the left frontal lobe and upper cervical spine. She was treated with intravenous immunoglobulins (IVIG), but symptoms returned several months later and then again. Subsequent improvements with therapy for ataxia, speech and feeding difficulties. Results: Overall cognitive functioning in the low average range with notable and extensive attentional limitations requiring continual prompting. Evaluation also revealed below grade and age level performance in all academic areas, significant oromotor and gross motor coordination difficulties, and mild self-esteem issues in this child who presented much younger than her chronological age. Conclusions: This case demonstrates the importance of neuropsychological evaluation in pediatric paraneoplastic motor/sensory motor syndromes. While underlying cause of demyelination and ataxia in this case remains unclear, cognitive findings revealed significant areas that require intervention which could have been identified and supported earlier in her development.