Gustafson L, Brun A, Passant U
Department of Psychogeriatrics, University of Lund, Sweden.
Baillieres Clin Neurol. 1992 Nov;1(3):559-82.
In a longitudinal prospective study of dementias, several hundred cases have been examined from a clinical, brain imaging, neurochemical and neuropathological point of view. Frontal lobe degeneration of non-Alzheimer type (FLD) was the second most common primary degenerative dementia found in about 10% of the material. FLD has a consistent pathology and a characteristic clinical picture, which have been described by several independent research groups. The cortical degeneration mainly involves frontal or frontotemporal grey matter, without the circumscribed or knife-blade atrophy seen in Pick's disease. The degeneration involves predominantly frontal areas, including the insula and cingulate gyrus in its anterior parts. The striate body is normal or only slightly altered. The pathological changes are non-specific, with neuronal loss, slight gliosis and spongiosis but none or few senile plaques, tangles, congophilic vessels or Pick cells. Pathological changes are in some respects similar to those in amyotrophic lateral sclerosis. FLD is a slowly progressive dementia with personality changes, lack of insight, disinhibition, stereotypy and later apathy. There is also progressive dynamic aphasia which ends in mutism and amimia. Memory, spatial ability and receptive language functions are comparatively spared. Psychotic symptoms, emotional reactions, hypochondriasis and a Klüver-Bucy-like syndrome are sometimes observed. Electroencephalography is normal, at least during the early stage, while functional brain imaging such as regional cerebral blood flow reflects the frontal pathology. It is possible to achieve early diagnosis and differentiation from Alzheimer's disease and cerebrovascular dementia by clinical examination with neuropsychological assessment supported by brain imaging, and in the future probably various biological markers. The aetiology is unknown but there is a positive family history for dementia of similar type in about 50% of post-mortem verified cases.
在一项关于痴呆症的纵向前瞻性研究中，已经从临床、脑成像、神经化学和神经病理学角度检查了数百个病例。非阿尔茨海默型额叶变性（FLD）是第二常见的原发性退行性痴呆，在约10%的病例中被发现。FLD具有一致的病理学特征和典型的临床表现，几个独立的研究小组都对此进行了描述。皮质变性主要累及额叶或额颞叶灰质，没有皮克病中所见的局限性或刀切样萎缩。变性主要累及额叶区域，包括岛叶和前扣带回。纹状体正常或仅有轻微改变。病理变化是非特异性的，有神经元丢失、轻度胶质细胞增生和海绵状变性，但没有或仅有少量老年斑、缠结、嗜刚果红血管或皮克细胞。病理变化在某些方面与肌萎缩侧索硬化症相似。FLD是一种缓慢进展的痴呆症，伴有性格改变、缺乏洞察力、脱抑制、刻板行为，后期出现淡漠。还存在进行性动态失语，最终导致缄默和模仿不能。记忆、空间能力和接受性语言功能相对保留。有时会观察到精神病症状、情绪反应、疑病症和克吕弗-布西样综合征。脑电图正常，至少在早期是这样，而功能性脑成像，如局部脑血流，反映了额叶病变。通过临床检查、神经心理学评估以及脑成像支持，未来可能还借助各种生物标志物，能够实现早期诊断并与阿尔茨海默病和脑血管性痴呆相鉴别。病因不明，但在约50%经尸检证实的病例中，有类似类型痴呆症的阳性家族史。
