Amano N, Matsuishi T, Suzuki K, Yokoi S, Saitoh A, Yagishita S
No To Shinkei. 1984 Jul;36(7):657-63.
This work is to study the ultrastructure of the neuronal lipofuscin that occurred in the brain and the spinal cord of an autopsy case of familial Alzheimer's disease and to compare with those in several other diseases. The patient was a 46-year-old male, whose father and elder brother were diagnosed as Alzheimer's disease and died at the age of 42, respectively. He became afflicted with forgetfulness and disorientation at the age of 36. He developed a grand mal seizure at the age of 39 and thereafter, his clinical course was characterized by pyramidal signs, dysarthria and the symptoms of Gerstmann's syndrome, visuo-spatial agnosia, apraxia for dressing and constructive apraxia. He became bedridden at 45 years old and died of general prostration. The brain weighed 1,250 g, and the cerebral cortex showed mild atrophy. The neuronal loss, senile plaques and Alzheimer's neurofibrillary tangles were found throughout the cerebral cortex. The senile plaques were also found in the basal ganglia, the cerebellar medulla and cortex. There was severe amyloid angiopathy in the occipital and cerebellar cortices. The specimens for electron microscopy were taken from the cerebral cortex, the basal ganglia, the thalamus, the midbrain, the medulla oblongata, the cerebellum and the spinal cord. The ultrastructural study revealed three different types of the neuronal lipofuscin, though different stainability between these lipofuscin granules could not be manifested by several histochemical methods. Their morphological differences seemed to be based on the sites of the central nervous system.(ABSTRACT TRUNCATED AT 250 WORDS)
本研究旨在探讨家族性阿尔茨海默病尸检病例脑和脊髓中出现的神经元脂褐素的超微结构,并与其他几种疾病中的情况进行比较。患者为46岁男性,其父亲和哥哥均被诊断为阿尔茨海默病,分别于42岁时去世。他36岁时开始出现健忘和定向障碍。39岁时发生大发作,此后其临床病程表现为锥体束征、构音障碍以及格斯特曼综合征的症状、视觉空间失认、穿衣失用和结构性失用。45岁时卧床不起,死于全身衰竭。脑重1250克,大脑皮质显示轻度萎缩。在整个大脑皮质均发现神经元丢失、老年斑和阿尔茨海默病神经原纤维缠结。在基底神经节、小脑髓质和皮质也发现了老年斑。枕叶和小脑皮质存在严重的淀粉样血管病。用于电子显微镜检查的标本取自大脑皮质、基底神经节、丘脑、中脑、延髓、小脑和脊髓。超微结构研究揭示了三种不同类型的神经元脂褐素,尽管几种组织化学方法无法显示这些脂褐素颗粒之间不同的染色性。它们的形态差异似乎基于中枢神经系统的部位。(摘要截选至250字)