Brito-Marques P R, Mello R V, Montenegro L
Behavioral Neurology Unit, Department of Neurology, Faculty of Medical Sciences, University of Pernambuco, Brazil.
Arq Neuropsiquiatr. 2001 Mar;59(1):128-33. doi: 10.1590/s0004-282x2001000100028.
We report on a patient presenting Pick's disease similar to the one reported by Pick in 1892, with ubiquitin-positive and tau-negative inclusions. His diagnosis was made on the basis of clinical (language disturbance and behavioural disorders), neuropsychological (progressive aphasia of the expression type and late mutism), neuroimaging with magnetic resonance (bilateral frontal and temporal lobes atrophy) and brain single photon emission computed tomography (frontal and temporal lobes hypoperfusion) studies. Macroscopic examination showed atrophy on the frontal and temporal lobes. The left hippocampus displayed a major circumscribed atrophy. The diagnostic confirmation was made by the neuropathological findings of the autopsy that showed neuronal loss with gliosis of the adjacent white matter and apearance of status spongiosus in the middle frontal and especially in the upper temporal lobes. There were also neuronal swelling (ballooned cell) and argyrophilic inclusions (Pick's bodies) in the left and right hippocampi. Anti-ubiquitin reaction tested positive and anti-tau tested negative.
我们报告了一例表现为类似1892年皮克所报告疾病的皮克病患者，其包涵体泛素阳性而tau阴性。他的诊断基于临床症状（语言障碍和行为紊乱）、神经心理学表现（表达型进行性失语和晚期缄默症）、磁共振神经影像学检查（双侧额叶和颞叶萎缩）以及脑单光子发射计算机断层扫描（额叶和颞叶灌注不足）研究。大体检查显示额叶和颞叶萎缩。左侧海马出现明显的局限性萎缩。尸检的神经病理学结果证实了诊断，结果显示神经元丢失，相邻白质胶质增生，额中回尤其是颞上回出现海绵状状态。左右海马还存在神经元肿胀（气球样细胞）和嗜银包涵体（皮克小体）。抗泛素反应呈阳性，抗tau反应呈阴性。
