Hishikawa N, Hashizume Y, Hirayama M, Imamura K, Washimi Y, Koike Y, Mabuchi C, Yoshida M, Sobue G
Department of Neurology, Nagoya University School of Medicine, Japan.
Clin Auton Res. 2000 Jun;10(3):139-43. doi: 10.1007/BF02278018.
The authors report an autopsy case characterized by progressive lethargy and autonomic failure with a distinctive pattern of occurrence of Lewy bodies. Autonomic dysfunction such as sleep apnea, orthostatic hypotension, dysuria, and hypohidrosis predominated with lethargy, whereas parkinsonism was not apparent. Numerous Lewy bodies were widely evident microscopically in brainstem nuclei and the intermediolateral cell columns of the spinal cord, as well as in the sympathetic ganglia, but were rare or absent in the cerebral cortex and other supratentorial structures. Marked neuronal loss was seen in the locus ceruleus, raphe nuclei, dorsal vagal nuclei, and intermediolateral cell columns, but neurons in the substantia nigra, other brain regions, and sympathetic ganglia appeared undiminished. This case represents a specific clinicopathologic form of Lewy body disease occurring predominantly in the brainstem, spinal cord, and sympathetic ganglia.
作者报告了一例尸检病例，其特征为进行性嗜睡和自主神经功能衰竭，并伴有路易小体独特的出现模式。以嗜睡为主的自主神经功能障碍，如睡眠呼吸暂停、体位性低血压、排尿困难和少汗较为突出，而帕金森综合征并不明显。在显微镜下，大量路易小体广泛见于脑干核、脊髓中间外侧细胞柱以及交感神经节，但在大脑皮质和其他幕上结构中则很少见或不存在。在蓝斑、中缝核、迷走神经背核和中间外侧细胞柱中可见明显的神经元丢失，但黑质、其他脑区和交感神经节中的神经元似乎未减少。该病例代表了一种主要发生在脑干、脊髓和交感神经节的路易体病的特定临床病理形式。
