Tsuchiya K, Ishizu H, Nakano I, Kita Y, Sawabe M, Haga C, Kuyama K, Nishinaka T, Oyanagi K, Ikeda K, Kuroda S
Department of Laboratory Medicine and Pathology, Tokyo Metropolitan Matsuzawa Hospital, Japan.
Acta Neuropathol. 2001 Nov;102(5):441-8. doi: 10.1007/s004010100388.
We investigated four Japanese autopsy cases of the generalized variant of Pick's disease ("basophilic inclusion body disease") both clinically and pathologically, and examined the degree and distribution of the basal ganglia lesions, including the amygdala, striatum, pallidum, and substantia nigra. The lesions in the amygdala, striatum, and pallidum were classified into three categories (slight, moderate, and severe). The lesions in the substantia nigra were qualitatively judged, compared with normal controls. Extrapyramidal signs, not noticed in the generalized variant of Pick's disease, were evident in all four cases, in addition to dementia. The degree and distribution of basal ganglia lesions in all four cases were uniform: the caudate nucleus showed severe lesions, the amygdala and putamen severe to moderate lesions, and the pallidum moderate to slight lesions. The substantia nigra in all our cases showed prominent neuronal loss, probably being one of the lesions responsible for extrapyramidal signs. In the generalized variant of Pick's disease, the degree and distribution of the alterations within the basal ganglia differs from those reported in Pick's disease with Pick bodies (PDPB) and corticobasal degeneration (CBD). In PDPB, severe lesions are present in the amygdala with relative sparing of the substantia nigra, compatible with rare extrapyramidal signs in PDPB, while in CBD, severe lesions are found in the pallidum and substantia nigra. These clinicopathological findings may contribute not only to the elucidation of clinicopathological hallmarks, but also to the progress of neuroimaging, in the generalized variant of Pick's disease.
我们对4例日本尸检确诊的匹克病广义变异型（“嗜碱性包涵体病”）患者进行了临床和病理研究，检查了基底神经节病变的程度和分布情况，包括杏仁核、纹状体、苍白球和黑质。杏仁核、纹状体和苍白球的病变分为三类（轻度、中度和重度）。将黑质病变与正常对照进行定性判断。除痴呆外，所有4例患者均出现了匹克病广义变异型中未发现的锥体外系症状。所有4例患者基底神经节病变的程度和分布均一致：尾状核显示重度病变，杏仁核和壳核显示重度至中度病变，苍白球显示中度至轻度病变。我们所有病例的黑质均显示出明显的神经元丢失，这可能是导致锥体外系症状的病变之一。在匹克病广义变异型中，基底神经节内改变的程度和分布与有匹克小体的匹克病（PDPB）和皮质基底节变性（CBD）报道的情况不同。在PDPB中，杏仁核存在重度病变，黑质相对 spared，这与PDPB中罕见的锥体外系症状相符，而在CBD中，苍白球和黑质存在重度病变。这些临床病理结果不仅可能有助于阐明临床病理特征，还可能有助于匹克病广义变异型神经影像学的进展。
