A Case of Acute Combined Central and Peripheral Demyelination

Abstract

Demyelinating disease can acutely affect the central or peripheral nervous system in the forms of acute disseminated encephalomyelitis, acute inflammatory demyelinating polyradiculoneuropathy, and related disorders. Rarely, demyelination can affect the central and peripheral nervous system simultaneously in a disorder that has been described as acute severe combined demyelination. We describe a 54-year-old woman who presented with rapidly progressive ascending paralysis and areflexia with abnormalities in cerebrospinal fluid, electrodiagnostic studies, and magnetic resonance imaging of the brain and cervical spine. She had antibodies to GD1a and responded to treatment with intravenous immunoglobulin and corticosteroids. Our case illustrates that, even in severe form, acute combined central and peripheral demyelination may be considered a treatment responsive and potentially reversible disease.