Sodeyama N, Shimada M, Uchihara T, Yanagisawa K, Fujigasaki H, Yamaguchi K, Matsushita M, Yamada M
Department of Neurology, Tokyo Medical and Dental University, Japan.
J Neurol Neurosurg Psychiatry. 1995 Oct;59(4):395-9. doi: 10.1136/jnnp.59.4.395.
Two sisters with familial Alzheimer's disease developed spastic gait disturbance as an initial manifestation. Their gait disturbance progressed gradually, followed by dementia a few years later. Post-mortem examination of one of the patients disclosed degeneration of the thalamus and corticospinal tract in addition to numerous senile plaques and neurofibrillary tangles in the neocortex, both of which were confirmed by immunohistochemistry. This is the first report in which clinicopathological evaluation is sufficient to establish a new variant of Alzheimer's disease presenting initially as spastic tetraplegia.
两名患有家族性阿尔茨海默病的姐妹最初表现为痉挛性步态障碍。她们的步态障碍逐渐进展，几年后出现痴呆。对其中一名患者进行的尸检发现，除了新皮质中有大量老年斑和神经原纤维缠结外，丘脑和皮质脊髓束也发生了退化，这两者均通过免疫组织化学得到证实。这是首份临床病理评估足以确立一种最初表现为痉挛性四肢瘫的阿尔茨海默病新变体的报告。
