Dystonia musculorum deformans alleviated by chemopallidothalamectomy and substantia nigralysis. Case report.

Abstract

Dystonia musculorum deformans, as the name implies, is generally a crippling and progressive neuromuscular disorder. I t results in grotesque postures and finally in permanent skeletal changes, such as rotokyphoscoliosis. I t is primarily a disorder of childhood and adolescence and therefore the more devastating. With the use of chemopallidectomy and chemothalamectomy introduced by Cooper] the majority of such patients can be greatly improved if not cured of their dystonic syndrome. In our 6 cases, 4 patients have been completely relieved and 1 considerably improved, and 1 has received no benefit from surgery. One patient required bilateral thalamic lesions which incidentally were done simultaneously; in a second patient, bilateral pallidal lesions were made at 4 separate operations; in a third, bilateral pallidal and unilateral thalamic lesions were produced in 3 operations; a fourth has had unilateral thalamic lesions on ~ occasions with 75 per cent improvement and soon will have a pallidal lesion made. The fifth patient has been the most difficult to treat, requiring bilateral pallidal and unilateral thalamic lesions done in 6 operations. And, in addition, destruction of the left substantia nigra by a new surgical technique-substantia nigralysis2 This particular case is worthy of detailed review.