Chronic inflammatory demyelinating polyneuropathy caused by HIV infection in a patient with asymptomatic CMT 1A

It is well known that patients with Charcot-Marie-Tooth (CMT) disease are l iable to present with episodes of cortisone-responsive demyelination, and a superimposed inflammatory component has been suggested. We report a patien t who presented with a chronic inflammatory demyelinating polyneuropathy du e to a recent HIV infection, which revealed a previously asymptomatic CMT 1 A disease documented by identification of the characteristic duplication on the p11.2 region of chromosome 17. The inflammatory process was characteri zed by pathologic findings on a superficial peroneal nerve biopsy, and the patient improved significantly after corticotherapy. This report gives supp ort to the hypothesis of a genetic susceptibility to inflammatory demyelina ting processes in certain CMT kindreds.