Acute relapse in CMT 1B neuropathy: can protein P0 behave like an autoantigen?

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Abstract from Rev Neurol (Paris). 2004 Sep;160(8-9):839-42.

Acute relapse in Charcot-Marie-Tooth 1B neuropathy: can protein P0

behave like an autoantigen?

Fleury M, Anheim M, Tranchant C, Warter JM.

Departement de Neurologie, Hopitaux Universitaires, Strasbourg.

Introduction. The natural history of Charcot-Marie-Tooth neuropathy is

marked by accentuated motor and sensitive deficits suggestive of acute

polyradiculoneuritis or, more generally, chronic inflammatory

demyelinizing polyneuropathy. Observation. A 41-year-old woman, with

Charcot-Marie-Tooth (CMT) 1B neuropathy associated with a P0 gene

mutation, developed several episodes of ataxia which resolved after

intravenous administration of IgG or corticosteroids.

CONCLUSION: The sudden increase of a motor or sensitive deficit in this

patient with CMT type I led to two hypotheses: chance association

between an inherited and an inflammatory neuropathy, or a dysimmune

inflammatory reaction, the mutated protein acting like an autoantigen.

These two hypotheses are discussed.