Kuzuhara S, Yoshimura M
Department of Neurology, Mie University School of Medicine, Japan.
Adv Neurol. 1993;60:464-9.
This chapter reports the clinical and neuropathological findings of eight cases of "diffuse Lewy body disease" verified by autopsy. The age at onset was between 60 and 82 years; the age at death was between 75 and 92 years. The initial symptoms were amnesia in three cases, orthostatic dizziness in three, visual hallucination in two, but parkinsonism in none. The cardinal clinical symptoms included dementia in all cases, hallucinatory-delusional state in six, akinesia and rigidity in five, and orthostatic hypotension in five. Antemortem diagnoses were senile dementia in five, and hallucinatory-delusional state, Parkinson's disease and Shy-Drager syndrome in one each. Despite the clinical symptoms differences from each other, neuropathological findings were alike. Abundant Lewy bodies were present in the neurons of the cerebral cortex as well as in the brainstem nuclei and diencephalon. Concomitant senile changes including senile plaques and Alzheimer's neurofibrillary tangles (NFTs) were also present in varying degree. Immunocytochemical study with anti-ubiquitin for Lewy body, anti-tau protein for NFT, and beta-protein of amyloid for senile plaque suggested that dementia of DLBD might have resulted not from a single pathology but from the complex of Lewy bodies, NFTs and senile plaques.
本章报告了8例经尸检证实的“弥漫性路易体病”的临床和神经病理学发现。发病年龄在60至82岁之间;死亡年龄在75至92岁之间。初始症状有3例为失忆,3例为体位性头晕,2例为视幻觉,但均无帕金森综合征。主要临床症状包括所有病例均有痴呆,6例有幻觉妄想状态,5例有运动不能和强直,5例有体位性低血压。生前诊断5例为老年性痴呆,1例为幻觉妄想状态,1例为帕金森病,1例为Shy-Drager综合征。尽管临床症状各不相同,但神经病理学发现相似。大脑皮质神经元以及脑干核和间脑均有大量路易体。同时还不同程度地存在包括老年斑和阿尔茨海默神经原纤维缠结(NFTs)在内的老年变化。用抗泛素检测路易体、抗tau蛋白检测NFT以及用淀粉样β蛋白检测老年斑的免疫细胞化学研究表明,DLBD痴呆可能并非由单一病理所致,而是由路易体、NFTs和老年斑共同作用的结果。