Kertesz A, Hudson L, Mackenzie I R, Munoz D G
Department of Clinical Neurological Sciences, St. Joseph's Hospital, London, ON, Canada.
Neurology. 1994 Nov;44(11):2065-72. doi: 10.1212/wnl.44.11.2065.
We present three cases of primary progressive aphasia (PPA) with Pick-variant pathology to support a hypothesis of an underlying nosologic relatedness. Neuropathologic examination demonstrated focal brain atrophy with corresponding neuronal loss and gliosis, accompanied by superficial spongiosis. Specific histologic findings were ballooned neurons (Pick cells) in the atrophic areas, and in two of the cases, Pick bodies. They were immunoreactive for tau. In contrast to classic Pick's disease, there were no Pick bodies in the hippocampus. The intense neurofilament immunoreactivity of the perikarya of the ballooned neurons greatly facilitated their recognition. Based on our cases and a critical review of the literature, we hypothesize that the common underlying pathology of PPA is a variant of Pick's disease. Furthermore, we propose the concept of "Pick complex" to include other neurodegenerative diseases characterized by focal cortical degeneration, such as PPA, frontal lobe dementia, ALS with PPA, and corticonigral and corticobasal ganglionic degenerations.
我们展示了三例具有Pick变异型病理特征的原发性进行性失语（PPA）病例，以支持一种潜在疾病相关性的假说。神经病理学检查显示局灶性脑萎缩，伴有相应的神经元丢失和胶质增生，并伴有浅层海绵状变性。具体的组织学发现为萎缩区域出现气球样神经元（Pick细胞），其中两例还出现了Pick小体。它们对tau蛋白呈免疫反应性。与经典的Pick病不同，海马体中没有Pick小体。气球样神经元胞体强烈的神经丝免疫反应性极大地有助于它们的识别。基于我们的病例以及对文献的批判性回顾，我们推测PPA的共同潜在病理是Pick病的一种变异型。此外，我们提出“Pick复合体”的概念，以包括其他以局灶性皮质变性为特征的神经退行性疾病，如PPA、额叶痴呆、合并PPA的肌萎缩侧索硬化症以及皮质黑质和皮质基底节变性。
