Rodda R A
J Neurol Sci. 1981 Apr;50(1):147-57. doi: 10.1016/0022-510x(81)90049-6.
The clinical features are outlined and the neuropathological changes described in 3 cases (2 adults and 1 child) of Huntington's disease with severe atrophy of the cerebellum. Onset occurred at the ages of 56, 55 and 3 and death at 70, 62 and 6 years, respectively. All cases presented with cerebellar ataxia and this is also recorded in one relative of each adult case. The family history of Huntington's disease was not ascertained until the later stages of each patient's illness. At necropsy, the 3 cases showed the characteristic striatal and cerebral cortex atrophy and the shrunken cerebellum showed diffuse thinning of the molecular and granular layers with almost complete loss of Purkynĕ cells.
本文概述了3例（2例成人，1例儿童）患有严重小脑萎缩的亨廷顿病患者的临床特征，并描述了其神经病理学变化。发病年龄分别为56岁、55岁和3岁，死亡年龄分别为70岁、62岁和6岁。所有病例均表现为小脑共济失调，且每例成年患者的一名亲属也有此症状。直到每位患者疾病的后期才确定其亨廷顿病家族史。尸检时，3例患者均表现出典型的纹状体和大脑皮质萎缩，萎缩的小脑显示分子层和颗粒层弥漫性变薄，浦肯野细胞几乎完全丧失。
