Chronic inflammatory demyelinating polyneuropathy presenting with headache and papilledema

Chronic inflammatory demyelinating polyneuropathy is a disorder typified clinically by motor and sensory neuropathy of at least 2 months' duration and pathologically by multifocal inflammatory demyelination. Its usual presentation is with features reflecting the polyneuropathy, namely limb weakness with hyporeflexia or areflexia and sensory symptoms of glove and stocking distribution. In this report, we detail the course of a 53-year-old man who presented to our neurological service with a severe headache in association with papilledema. The initial diagnosis considered was of possible primary intracranial pathology. Two months later, he developed limb weakness and sensory symptoms typical of chronic inflammatory demyelinating polyneuropathy. His headache, papilledema, and limb symptoms responded to oral corticosteroid therapy, the standard treatment for this type of neuropathy. We hypothesize that his headache and papilledema were due to the elevated cerebrospinal fluid protein level as a result of the polyneuropathy. To our knowledge, this is the first report of headache being a prominent and early symptom of this disorder.