Brown J, Lantos P, Stratton M, Roques P, Rossor M
Department of Neurology, St Mary's Hospital, London.
J Neurol Neurosurg Psychiatry. 1993 May;56(5):473-6. doi: 10.1136/jnnp.56.5.473.
A progressive extrapyramidal syndrome and dementia occurred in three members of one family. The age of onset was in the seventh decade and the affected individuals showed many of the clinical features of progressive supranuclear palsy (PSP). Necropsy of one individual revealed the neuropathological features of PSP. We propose that this family has a familial form of PSP and review the evidence in the literature that a familial form exists.
一个家族的三名成员出现了进行性锥体外系综合征和痴呆。发病年龄在七十多岁,患病个体表现出许多进行性核上性麻痹(PSP)的临床特征。对其中一名个体进行尸检,发现了PSP的神经病理学特征。我们认为这个家族患有家族性PSP,并回顾了文献中关于家族性形式存在的证据。