Katayama S, Watanabe C, Khoriyama T, Oka M, Mao J J, Yamamura Y, Tahara E, Nakamura S
Third Department of Internal Medicine, Hiroshima University School of Medicine, Japan.
J Neurol Sci. 1998 Dec 11;161(2):169-72. doi: 10.1016/s0022-510x(98)00278-0.
We report an autopsy case of a 51-year old man who showed slowly progressive pure akinesia: freezing phenomenon and festination during 21 years of illness without tremor, rigidity, upward gaze palsy, bradykinesia and dementia, which were not responded to L-DOPA clinically. Neuropathological findings revealed the circumscribed regions in the substantia nigra and middle portion of the internal globus pallidus (GPi), without neurofibrillary tangles, neuropil threads, and glial fibrillary tangles. So this case was clearly distinguished with progressive nuclear palsy and pallidonigroluisian atrophy. It was first reported to describe that L-DOPA nonresponsive pure akinesia can arise from nigopallidal atrophy.
我们报告一例51岁男性的尸检病例，该患者表现为缓慢进展的单纯运动不能：在21年病程中出现冻结现象和慌张步态，无震颤、强直、向上凝视麻痹、运动迟缓及痴呆，临床对左旋多巴无反应。神经病理学检查发现黑质和苍白球内侧部（GPi）有局限性区域，无神经原纤维缠结、神经毡丝和胶质原纤维缠结。因此，该病例与进行性核上性麻痹和苍白球黑质路易体萎缩明显不同。首次报道描述了左旋多巴无反应的单纯运动不能可由黑质苍白球萎缩引起。
