Odawara T, Iseki E, Yagishita S, Amano N, Kosaka K, Hasegawa K, Matsuda Y, Kowa H
Department of Psychiatry and Neurology, Yokohama City University School of Medicine, Japan.
Clin Neuropathol. 1992 May-Jun;11(3):131-4.
An autopsied case of juvenile parkinsonism and dementia is described. The patient is a 48-year-old man who had a ten-year history of parkinsonian syndrome and progressive dementia. Neuropathological examination revealed a widespread occurrence of Lewy bodies and spheroids in the central nervous system. Lewy bodies were found not only in the brain stem and diencephalon, but also in the cerebral cortex. Massive numbers of small spheroids were observed in the globus pallidus, substantia nigra, mamillary bodies and hippocampus. Electron microscopical examination showed that most spheroids were composed of degenerative organelles with only a few neurofilaments, and were different from those of Hallervorden-Spatz disease. There was also marked neuronal loss with gliosis in the CA3-4 of the hippocampus. Some neurofibrillary tangles occurred in the hippocampus, subcortical and brain stem nuclei, but senile plaques were absent. This case may represent an atypical form of pure diffuse Lewy body disease.
本文描述了一例青少年帕金森病合并痴呆的尸检病例。患者为一名48岁男性，有十年帕金森综合征和进行性痴呆病史。神经病理学检查显示中枢神经系统广泛出现路易小体和球形小体。路易小体不仅见于脑干和间脑，还见于大脑皮层。在苍白球、黑质、乳头体和海马中观察到大量小的球形小体。电子显微镜检查显示，大多数球形小体由退化的细胞器组成，只有少数神经丝，与Hallervorden-Spatz病的球形小体不同。海马CA3-4区也有明显的神经元丢失和胶质细胞增生。海马、皮层下和脑干核中出现了一些神经原纤维缠结，但无老年斑。该病例可能代表了一种非典型的纯弥漫性路易体病形式。
