[Dentatorubropallidoluysian atrophy (DRPLA): comparative pathological study on clinical groups classified into juvenile, early adult and late adult types].

We performed a clinicopathological study on 12 autopsied cases of dentatorubropallidoluysian atrophy. They were divided into 3 groups according to the age at onset: juvenile type (6 cases), early adult type (4 cases) and late adult type (2 cases). In juvenile type showing progressive myoclonus epilepsy (PME) syndrome, degeneration of the globus pallidus was more marked than that of the dentate nucleus. Mild to moderate atrophy was seen in the brain stem and spinal cord. In early adult type showing milder symptom of myoclonus and epilepsy, the globus pallidus and dentate nucleus were equally degenerated to various extents in most cases. Atrophy of the brain stem and spinal cord was mild to moderate in degree. In late adult type without PME syndrome, degeneration of the dentate nucleus was more marked than that of the globus pallidus. The brain stem and spinal cord were severely atrophic. On the other hand, the cases showing severe dentate lesion had a tendency to show severe atrophy of the brain stem and spinal cord. We consider that development of myoclonus, epilepsy and choreoathetoid movement in DRPLA patients has close relation to the extent of not only degeneration of the globus pallidus and dentate nucleus, but also atrophy of the brain stem and spinal cord.