Selective IgA deviciency in Charcot-Marie-Tooth disease.

A selective IgA deficiency was found in five of 15 random patients with Charcot-Marie-Tooth disease. Immunologic studies of 32 members of the five families showed low IgA in 17 of the members, associated with elevated IgM in 20, without other significant immune abnormalities. The immunoglobulin pattern and the variability of symptoms in this familial peripheral neuropathy suggest that a thymic or gut-associated immune deficiency or both, may be causally related to the disease process.