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[一例帕金森病尸检病例,临床伴有痴呆,最终发展为运动不能性缄默症,病理检查发现大脑皮质有多个路易小体]

[An autopsy case of Parkinson's disease associated clinically with dementia terminating in akinetic mutism and pathologically with multiple Lewy's Bodies in the cerebral cortex].

作者信息

Okeda R, Kayano T, Funata N, Kojima T, Miki M, Iwama H

出版信息

No To Shinkei. 1982 Aug;34(8):761-7.

PMID:6289855
Abstract

An autopsy case of a 50 year-old male with Parkinson's disease associated with multiple Lewy bodies in the cerebral cortex was reported. His clinical symptoms began at the age of 26 with the speech and actions indicative of a persecution complex accompanied by irritability and were followed by progressive dementia from the age of 37 and Parkinsonism since the age of 41. He was in a state of akinetic mutism thereafter till his death at the age of 50. Autopsy disclosed in addition to the typical findings of Parkinson's disease in the brain stem multiple intracytoplasmic Lewy bodies in medium-sized neurons of the fifth and sixth layers of the cerebral cortex. They were atypical in the sense that they did not have any haloes. They were especially numerous in the cingulate gyrus. In addition, findings of non-specific neuronal degeneration were obtained in the cerebral cortex such as cellular atrophy with massive deposition of lipofuscin pigments, central chromatolysis, cell loss and cellular gliosis in the third, fifth and sixth layers. These neuronal findings were also prominent in the cingulate gyrus. Such senile changes as senile plaques or granulo-vacuolar degeneration were not found, although there were a few foci of neurofibrillary degeneration in the hippocampal gyrus. Histochemically and electron microscopically, no difference was observed in the constituents of Lewy bodies between the brain stem and the cerebral cortex. Such autopsy findings and a review of the literature indicate that the dementia in this case may be related not only to the presence of Lewy bodies but also to the above-described, non-specific neuronal degeneration in the bilateral cingulate gyri and surrounding frontal gyri. The standpoint of regarding a Parkinson's disease with multiple Lewy bodies in the cerebral cortex as an independent disease entity was criticized.

摘要

报告了一例50岁男性帕金森病尸检病例,其大脑皮质存在多个路易小体。他的临床症状始于26岁,表现为言语和行为显示被迫害妄想,伴有易怒,随后从37岁开始出现进行性痴呆,41岁起出现帕金森综合征。此后他一直处于运动不能性缄默状态,直至50岁去世。尸检发现,除脑干有帕金森病的典型表现外,大脑皮质第五和第六层中型神经元内有多个胞浆内路易小体。这些路易小体不典型,因为它们没有任何晕环。它们在扣带回中尤其多。此外,在大脑皮质还发现了非特异性神经元变性的表现,如细胞萎缩伴脂褐素大量沉积、中央性染色质溶解、细胞丢失以及第三、第五和第六层的细胞胶质增生。这些神经元表现在扣带回中也很突出。尽管海马回有一些神经原纤维变性灶,但未发现老年斑或颗粒空泡变性等老年变化。组织化学和电子显微镜检查显示,脑干和大脑皮质的路易小体成分没有差异。这些尸检结果及文献回顾表明,该病例中的痴呆可能不仅与路易小体的存在有关,还与双侧扣带回及周围额叶上述非特异性神经元变性有关。将大脑皮质有多个路易小体的帕金森病视为一种独立疾病实体的观点受到了批评。

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