Idiopathic dystonia musculorum deformans

Abstract

Attention is directed to the formes frustes of dystonia musculorum deformans as found in 4 generations of 1 family with this genetic disorder. These persons lack the typical dystonic movements and postures. Their manifestations are chiefly dyssynergias, hyperkinesias not commonly related to dystonia, postural abnormalities and contracture deformities not associated with dystonic hyperkinesias, and, possibly, also mental deficiency. It is suggested that, at least in some instances, the formes frustes may be prematurely arrested cases of dystonia. The importance of the recognition of formes frustes as regards treatment, prognosis, and eugenic implications is stressed.