A Case of Hereditary Progressive Dystonia With Diurnal Fluctuation

ABSTRACT

Hereditary progressive dystonia develope in the first decade of ?life and is characterized by marked diurnal fluctuation of symptoms(dystonic postures and movements), i.e.symptoms aggrevated towards evening and alleviated after sleep. These symptoms and signs responded dramatically to levodopa. With the high incidence of familial cases this disorder is considers to be hereditary, but inheritance is not clear yet. Segawa regarded this disorder as a dystonia different from Parkinson's disease and other types of dystonic movement disorders, thereafter it has been reported under name of hereditary progressive dystonia with marked diurnal fluctuation.