A rare cause of gait ataxia

Diagnosis was confirmed by biopsy of ear cartilage; the sample showed necrosis of the cartilage matrix and perichondral inflammation with lymphocytic and plasmocytic infiltrate-a pattern that is quite specific to the disease.2 Retrospectively, ear oedema was visible on diffusion MRI (figure B). By c...

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Bibliographic Details
Published in:The Lancet (British edition) 2011-10, Vol.378 (9798), p.1274-1274
Main Authors: Roux, Charles, MD, Guey, Stéphanie, MD, Crassard, Isabelle, MD, Hautefort, Charlotte, MD, Lioté, Frédéric, MD, Jouvent, Eric, Dr
Format: Article
Language:English
Subjects:
Ataxia
Biological and medical sciences
Ear Cartilage - pathology
Ears & hearing
Gait Ataxia - etiology
General aspects
Hearing Loss, Sensorineural - complications
Humans
Internal Medicine
Male
Medical diagnosis
Medical imaging
Medical sciences
Middle Aged
Mortality
Nervous system (semeiology, syndromes)
Nervous system as a whole
Neurology
Polychondritis, Relapsing - complications
Polychondritis, Relapsing - diagnosis
Polychondritis, Relapsing - drug therapy
Polychondritis, Relapsing - pathology
Vertigo
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Summary:Diagnosis was confirmed by biopsy of ear cartilage; the sample showed necrosis of the cartilage matrix and perichondral inflammation with lymphocytic and plasmocytic infiltrate-a pattern that is quite specific to the disease.2 Retrospectively, ear oedema was visible on diffusion MRI (figure B). By contrast to auditory loss, vestibular dysfunction is rare in relapsing polychondritis and was the initial symptom in only one of 23 cases.3 Usual manifestations are auricular and nasal chondritis, arthritis, ocular inflammation, and laryngotracheal symptoms before vestibulocochlear dysfunction.2 In relapsing polychondritis, vestibular dysfunction can be secondary to chondritis-induced destruction of the eustachian tube leading to inflammation of the inner ear.
ISSN:0140-6736
1474-547X
DOI:10.1016/S0140-6736(11)61024-6