[Dystonia preceding dopa-responsive parkinsonism--heterogeneous clinical features]

We report six patients with dystonia-parkinsonism syndrome having a common characteristic clinical course of dystonia with subsequent development of levodopa-responsive parkinsonism. Various types of dystonic symptoms were observed such as hemidystonia, torticollis, axial dystonia, focal dystonia, or cranial dystonia (Meige's syndrome). Intervals between the onset of dystonia and that of parkinsonism varied from one year to about 20 years or the more. Levodopa and a dopamine against had inconstant effects on their dystonic symptoms except for the torticollis of the patients; on the other hand, parkinsonism were well relieved by levodopa in all of them. Dystonic symptoms showed heterogeneous drug effects. Carbamazepine had a beneficial effect on hemidystonia in a patient with hemidystonia-parkinsonism. In a patient with an unilateral hand cramp-ipsilateral parkinsonism, the focal dystonic symptom was evoked by cigarette smoking. Meige's syndrome became worse by the administration of levodopa. In contrast, in a patient with younger onset toriticollis-parkinsonism, both symptoms were fully relieved by levodopa. From the clinical and pharmacological points of views, this type of dystonia-parkinsonism, characterized by dystonia with later development of dopa-responsive parkinsonism, is a syndrome showing heterogeneous dopa-response, and it remains uncertain, at the present, that whether or not patients reported here share the same clinicopathological background such as striatal dopamine deficiency.