Schmitt H P, Yang Y, Förstl H
Institut für Neuropathologie, Universität Heidelberg, Germany.
Eur Arch Psychiatry Clin Neurosci. 1995;245(6):299-305. doi: 10.1007/BF02191871.
We report six cases of presenile (five) and senile (one) progressive dementia with a mild-to-marked frontal or frontotemporal atrophy and ventricular dilation (Frontal Lobe Degeneration [FLD]). The most prominent microscopic features were layer-dependent neuronal depletion of the cortex, spongiosis, and cortical and subcortical gliosis. Five cases showed additional degeneration of the S. nigra, and two also had motor neuron disease. Despite the absence of Pick cells and bodies, such cases have many features in common with Pick atrophy. Because Pick cells and bodies are inconstantly occurring features in otherwise typical cases of Pick atrophy, they cannot be regarded as inevitable markers of the latter. In our opinion, cases with mild frontal or frontotemporal atrophy as described herein and by others match the grades 1 and 2 in terms of Schneider's classification of Pick atrophy [37]. As long as the etiology of both Pick atrophy and the so-called FLD is unknown, and we finally have to follow morphological criteria for classification, there is apparently no convincing reason to introduce a separate category, such as FLD or FTA, for the cases with moderate or mild frontal atrophy and dementia of frontal lobe type, which can be sufficiently classified with the Pick spectrum of lobar atrophy.
我们报告了6例早老性（5例）和老年性（1例）进行性痴呆病例，伴有轻度至显著的额叶或额颞叶萎缩及脑室扩张（额叶变性[FLD]）。最突出的微观特征是皮质的层依赖性神经元缺失、海绵状变性以及皮质和皮质下胶质细胞增生。5例显示黑质额外变性，2例还患有运动神经元病。尽管没有匹克细胞和匹克小体，但这些病例与匹克萎缩有许多共同特征。因为匹克细胞和匹克小体在其他典型的匹克萎缩病例中并非恒定出现的特征，所以它们不能被视为后者的必然标志物。我们认为，本文及其他人所描述的伴有轻度额叶或额颞叶萎缩的病例，在施奈德匹克萎缩分类[37]中符合1级和2级。只要匹克萎缩和所谓的FLD的病因不明，而且我们最终必须遵循形态学标准进行分类，那么对于伴有中度或轻度额叶萎缩及额叶型痴呆的病例，显然没有令人信服的理由引入一个单独的类别，如FLD或额颞叶萎缩（FTA），因为它们可以用叶萎缩的匹克谱系进行充分分类。
