Chokroverty S, Nicklas W, Miller D C, Goldberg J, Choe J, Banner C, Thomas J, Duvoisin R
Department of Neurology, Robert Wood Johnson Medical School, New Brunswick, New Jersey.
J Neurol Neurosurg Psychiatry. 1990 Dec;53(12):1099-101. doi: 10.1136/jnnp.53.12.1099.
The neuropathological findings in a patient with antemortem diagnosis of olivopontocerebellar atrophy (OPCA) and reduced leucocytic glutamate dehydrogenase (GDH) activity included cerebellar cortical degeneration, most marked in the superior vermis, mild atrophy of the pons and the inferior olivary nucleus, marked reduction of anterior horn cells at all levels and gliosis in both lateral columns. GDH activities and their thermolability in "soluble" and "particulate" fractions in the cerebral cortex, cerebellar hemisphere and vermis were not significantly different from the values in two control brains. GDH mRNA in the patient's brain was not altered in size or amount.
一名生前诊断为橄榄体脑桥小脑萎缩（OPCA）且白细胞谷氨酸脱氢酶（GDH）活性降低的患者，其神经病理学发现包括小脑皮质变性，最明显的是在小脑上蚓部，脑桥和下橄榄核轻度萎缩，各级前角细胞明显减少以及双侧侧柱胶质增生。大脑皮质、小脑半球和蚓部“可溶性”和“颗粒性”部分中的GDH活性及其热稳定性与两个对照脑的值无显著差异。患者脑中的GDH mRNA在大小或数量上没有改变。
