Miyamoto K, Ikemoto A, Akiguchi I, Kohno R, Imura T, Fujita Y
Department of Neurology, Kyoto University, Japan.
Clin Neuropathol. 2001 Jan-Feb;20(1):8-12.
We report a patient with frontotemporal degeneration and parkinsonism with mental retardation. The patient was a 54-year-old man who had parkinsonism that resembled progressive supranuclear palsy, frontotemporal degeneration and myoclonus. His family included many affected members. Neuropathologically, there was degeneration of the frontal and temporal cortices, the basal ganglia, the brainstem and the cerebellum. Microscopically, neuronal loss was severe in the frontal and temporal cortex, the globus pallidus, substantia nigra, red nucleus and dentate nucleus. Fibrillary changes were found in neurons and glia that were immunostained for tau. Although we could not define the genetic abnormalities, we thought that this case might have involved frontotemporal dementia and parkinsonism linked to chromosome 17.
我们报告了一例患有额颞叶变性、帕金森症及智力发育迟缓的患者。该患者为一名54岁男性，患有类似进行性核上性麻痹、额颞叶变性及肌阵挛的帕金森症。其家族中有许多患病成员。神经病理学检查发现，额叶和颞叶皮质、基底神经节、脑干及小脑均有变性。显微镜下，额叶和颞叶皮质、苍白球、黑质、红核及齿状核神经元严重缺失。在经tau免疫染色的神经元和胶质细胞中发现了纤维状改变。尽管我们无法确定基因异常情况，但认为该病例可能与17号染色体相关的额颞叶痴呆和帕金森症有关。
