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A Course Over 20 Years With Cerebral Involvement

Alfred Dreher, MD; Joachim Aigner, PhD; Sabine Fuchshuber, MD; Ernst Kastenbauer, MD

Arch Otolaryngol Head Neck Surg. 2000;126:1495-1498.

Relapsing polychondritis is a chronic inflammatory disease associated with an autoimmune disorder in cartilaginous tissue, eyes, labyrinth, blood vessels, and central nervous system. We describe a 75-year-old woman who presented with a 20-year history of dyspnea, inspiratory stridor, and polyarthritis. She developed dysmorphism of both ears and a saddle nose approximately 10 years earlier. Subsequently, she suffered from hearing loss and a tremor. A T2-weighted magnetic resonance imaging scan of the brain revealed multiple, spotted signal intensities. Immunohistochemical analysis of a serum sample showed antibodies to cartilaginous tissue, which were further identified on immunoblotting as antibodies to type II collagen. The extremely prolonged course of disease (>20 years) until a correct diagnosis was made is remarkable. Also, cerebral involvement, which was most likely caused by cerebral angiitis, and which, to our knowledge, has never previously been reported in this form, was detected.

From the Department of Otorhinolaryngology, Ludwig-Maximilians-University, Munich, Germany.

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