Brun A
Department of Neuropathology, University Hospital, Lund, Sweden.
Arch Gerontol Geriatr. 1987 Sep;6(3):193-208. doi: 10.1016/0167-4943(87)90021-5.
Among 158 cases of organic dementia in a prospective study concerning both psychiatry and regional cerebral blood flow there were 26 cases with a mainly frontal or fronto-temporal dementia. Careful neuropathological investigation disclosed 20 cases of a mainly frontal or fronto-temporal grey matter degeneration, in four of them compatible with Pick's disease (2.5%) whereas 16 cases (10%) appeared to form a separate group without histological Alzheimer features and therefore named 'frontal lobe degeneration of non-Alzheimer type' (FLD). The remaining group of dementias of a clinically similar type proved to consist of cases of Jakob-Creutzfeldt's and Alzheimer's disease with frontal predominance and also a case of normal frontal cortex with a projected dysfunction caused by bilateral thalamic infarctions. Also other similar conditions are accounted for from the literature. The validity of the pathological changes described here, particularly with regard to their severity and regional distribution as well as their tendency to spare certain areas is attested by the clinical picture including neuropsychiatric symptoms and the regional cerebral blood flow pattern, both consistently producing the picture of a frontal and fronto-temporal disease of a progressive degenerative type. These features are dealt with in the following papers by Gustafson (1987) and Risberg (1987). FLD is in some morphological respects similar to other dementing disorders such as the ALS dementia complex and progressive subcortical gliosis, though with both clinical and clear-cut pathoanatomical differences. For the time being it seems safest to conclude that we are faced with a hitherto not fully recognized if not a new type of dementia caused by 'simple' neuronal degeneration of mainly the frontal or frontal and temporal lobes. It makes up about 10% of organic dementias, a figure that would be higher in purely clinical classifications due to the admixture of other frontal lobe disorders or frontally projected dysfunction clinically simulating FLD of the pathoanatomical type here described.
在一项关于精神病学和局部脑血流量的前瞻性研究中，158例器质性痴呆患者中有26例主要表现为额叶或额颞叶痴呆。仔细的神经病理学检查发现，20例主要为额叶或额颞叶灰质变性，其中4例符合匹克病（2.5%），而16例（10%）似乎构成一个单独的组，无组织学上的阿尔茨海默病特征，因此命名为“非阿尔茨海默型额叶变性”（FLD）。其余临床类型相似的痴呆组经证实包括以额叶为主的雅各布 - 克雅氏病和阿尔茨海默病病例，以及一例因双侧丘脑梗死导致预计功能障碍但额叶皮质正常的病例。文献中也提到了其他类似情况。这里描述的病理变化的有效性，特别是关于其严重程度、区域分布以及 spared某些区域的倾向，通过临床症状包括神经精神症状和局部脑血流模式得到了证实二者均一致呈现出进行性退行性类型的额叶和额颞叶疾病的表现。古斯塔夫森（1987年）和里斯伯格（1987年）在以下论文中论述了这些特征。FLD在某些形态学方面与其他痴呆性疾病如肌萎缩侧索硬化痴呆综合征和进行性皮质下胶质增生相似，尽管在临床和明确的病理解剖学上存在差异。目前看来最稳妥的结论是，我们面对的是一种迄今尚未被充分认识的，如果不是新型的，由主要是额叶或额叶和颞叶的“单纯”神经元变性引起的痴呆。它约占器质性痴呆的10%，由于其他额叶疾病或临床上模拟此处描述的病理解剖学类型的FLD的额叶投射功能障碍的混合，在纯临床分类中这一数字会更高。
