Relapsing polychondritis (RP) is a rare, recurrent, idiopathic autoimmune inflammation of cartilage including the nose, auricles, larynx, and trachea. Less than 3% of all RP present with neurologic complications, most commonly cranial neuropathies. Headaches, seizures, cerebellar ataxia, aneurysms, and aseptic meningitis have been described .
A previously healthy 60 year old man presented with 2 months of progressive headaches, cognitive decline, visual hallucinations, paranoid delusions, emotional lability and a 30 pound weight loss. He was alert, oriented to self and place, but had intermittent somnolence and agitation. He had a paucity of language with paraphasic errors. Strength and sensation were normal but reflexes were pathologically brisk. Intermittent twitching on his left face and diffuse action induced myoclonus were captured on video-EEG without underlying ictal activity.