Prolonged Guillain-Barre´ Syndrome: longstanding demyelination and axonopathy

Monophasic Guillain-Barré syndrome (GBS) of subtype AIDP (acute inflammatory demyelinating polyradiculoneuropathy) frequently renders prolonged disability. However, the neuropathic status of disabled GBS persons during later phases of their illness is less often explored. Attention instead focuses on acute immunomodulatory therapy early in their course. Here we highlight an index patient with protracted symptoms of AIDP studied over 46 months following the acute phase of her disease. Her findings are considered in the context of a further, retrospective cohort of 15 selected monophasic AIDP patients with prolonged disability. Serial clinical and electrophysiological assessments over 85 days indicated that electrophysiological features of ongoing demyelination accompanying axon damage are persistent, and often can suggest ongoing progression long after the acute phase of their illness. Current practice ascribes disability to bystander axon damage despite AIDP's designation as primarily demyelinating. However, chronically demyelinated axons may be at risk for later axon degeneration. Persons with prolonged courses of GBS may require newer therapeutic targets directed toward remyelination and axon regeneration.