Dystonia musculorum deformans, or torsion dystonia, originally was regarded as hysteria by Schwalbe.1 Ziehen2 described a case, calling the disorder spasm neurosis. Oppenheim,3 who added four cases, was the first to consider the disease organic. Mendell,4 without histopathologic proof, postulated possible lesions in the lenticular nucleus and brachium conjunctivum. The histopathologic reports in a few instances added little to the exact understanding of the disorder. The confusion arose mainly because of the difficulty in differentiating this condition from other dyskinesias, such as progressive hepatolenticular degeneration and chorea. Some observers have been inclined to consider dystonia musculorum deformans as a variant of hepatolenticular degeneration rather than as a disease entity. The constant histopathologic changes in the striatum and the dentate nucleus in our four cases, in which the patients were observed clinically for several years, may throw some light on the understanding of this disease.
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