GOLGI-KOPSCH SILVER STUDY OF THE BRAIN OF A PATIENT WITH UNTREATED PHENYLKETONURIA, SEIZURES, AND CORTICAL BLINDNESS
GOLGI-KOPSCH SILVER STUDY OF THE BRAIN OF A PATIENT WITH UNTREATED PHENYLKETONURIA, SEIZURES, AND CORTICAL BLINDNESS
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DOI：
10.1002/ajmg.1320440412
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发表时间：
1992-11-01
期刊：
影响因子：
--
通讯作者：
HEGSTRAND, L
中科院分区：
文献类型：
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作者：
KORNGUTH, S;GILBERTBARNESS, E;HEGSTRAND, L
本报告描述了一个未经治疗的27岁男性苯丙酮尿症，皮质盲，癫痫发作的大脑中观察到的形态变化。Golgi-Kopsch银、甲酚紫、苏木精和伊红染色用于研究小脑、外侧膝状体核、视觉皮层、额叶皮层和海马的细胞结构和组织。广泛的神经元损失发生在右侧膝状体核（LGN），视觉皮层和海马。左侧LGN，小脑和额叶皮质保留神经元成分; PKU受试者浦肯野细胞上的树突状突起数量减少。LGN和枕叶皮质神经元的丢失与失明有关，海马神经元的丢失与癫痫发作活动有关。
This report describes the morphological changes observed in the brain of an untreated 27-year-old man with phenylketonuria, cortical blindness, and seizures. Golgi-Kopsch silver, cresyl violet, and hematoxylin and eosin stains were used to study cell structure and organization of the cerebellum, the lateral geniculate nuclei, the visual cortex, frontal cortex, and hippocampus. Extensive neuronal losses occurred in the right lateral geniculate nucleus (LGN), the visual cortex, and hippocampus. The left LGN, cerebellum, and frontal cortex retained neuronal components; there was a reduction in the number of dendritic processes on the Purkinje cells of the PKU subject. The loss of neurons in the LGN and occipital cortex is related to the blindness and the neuronal loss in the hippocampus is related to seizure activity.