Inflammatory demyelination in a patient with CMT1A REPOST

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Muscle Nerve. 2003 Sep;28(3):373-6.

Inflammatory demyelination in a patient with CMT1A.

Vital A, Vital C, Lagueny A, Ferrer X, Ribiere-Bachelier C, Latour P,

Petry KG.

Department of Neuropathology, BP42, Victor Segalen University, 146

rue Leo-Saignat, 33076 Bordeaux, France.

We report a case of Charcot-Marie-Tooth disease (CMT), with

identified PMP22 gene duplication (CMT type 1A), and with evidence of

an inflammatory demyelinating process superimposed on the course of

the chronic genetic disease. Macrophage-associated demyelination was

observed on the peripheral nerve biopsy.

This observation supports some experimental data from the literature

and shows that there may be a genetic susceptibility to inflammatory

demyelinating processes in certain CMT kindreds.