Takahata N, Ito K, Yoshimura Y, Nishihori K, Suzuki H
Neurology. 1978 Sep;28(9 Pt 1):913-9. doi: 10.1212/wnl.28.9.913.
A familial disorder was characterized by chorea, ataxia, myoclonus, convulsions, dementia, and mental retardation. In five cases, the main lesion affected cerebellar dentate nuclei, with nerve cell loss, gliosis, chromatolysis, and grumose degeneration. Fibrous glial cell proliferation was detected in the globus pallidus.
一种家族性疾病的特征为舞蹈症、共济失调、肌阵挛、惊厥、痴呆和智力发育迟缓。在5例病例中,主要病变累及小脑齿状核,出现神经细胞丢失、神经胶质增生、染色质溶解和颗粒样变性。在苍白球中检测到纤维性神经胶质细胞增生。