额叶型痴呆的临床和病理

目的　证实一种少见的伴有痴呆的神经系统变性疾病———额叶型痴呆。方法　对 1例因并发肺炎而死亡的 46岁进行性痴呆患者 ,进行脑部剖检 ,经系列的组织染色及PrP ,tau蛋白等免疫组织化学染色。结果　该例患者有 :(1)进行性神经、精神症状 ,病程为 3年 ;(2 )头部CT示双侧额叶灰质萎缩 ,脑电图呈阵发性全导联 ,长间歇期 (>2s)的高波幅慢波 ;(3)脑重 10 5 0g ,脑萎缩仅限于额叶 ,未累及颞叶 ;(4)额叶灰质从第二层开始神经细胞大量脱失伴明显胶质增生 ,而锥体细胞相对完好。Beilschowsky及Gallyas染色无异常发现 ;(5 )神经细胞及胶质细胞内未发现任何包涵体 ;(6 )PrP、tau蛋白免疫组织化学染色呈阴性反应。结论　该病例为典型额叶型痴呆 ,今后在分析伴有痴呆的神经系统变性疾病时 ,应想到此类型痴呆。

Clinical and pathologpcal studies on Frontal lobe dementia

Objective To verify a uncommon neurodegenerative disease accompanying with dementia--frontal lobe dementia, or dementia of frontal lobe type (DFT). Methods A brain sample was obtained from a patient of 46-year-old male with progressive dementia. Conventional neurohistopathological examination and immunostaining for prion protein (PrP) and tau protein were performed, and clinical data were analysed. Results (1) It was shown having progressive neurological and psychical symptoms and a three month illness duration. (2) Atrophy was found in bilateral frontal gray matter in CT scan. Slow waves of high amplitude with long intermission of two second in whole course of electroencephalography examination were seen. (3) Brain weight was 1 050 g. The cerebral cortex was atrophied and restricted to frontal lobes. The temporal lobes were unaffected. (4) A severe loss of nerve cells from second frontal cortical layer with glioses was revealed, but pyramidal cells in this region remained intact. There were no positive findings on staining of Beilschowky and Gallyas methods. (5) No inclusions were seen in remaining nerve cells and gliocyte. (6) Immunohistochemistry revealed no significant changes on PrP and tau protein.Conclusion This is more typical a case of DFT , and now increasingly recognized. It suggests that a dementia of frontal lobe type should be considered when differential diagnosis of neurodegenerative disease with dementia have been made.