De Reuck Jacques, Caparros-Lefebvre Dominique, Deramecourt Vincent, Defebvre Luc, Auger Florent, Durieux Nicolas, Bordet Regis, Pasquier Florence, Maurage Claude-Alain
J. De Reuck, MD, PhD, Leopold II laan 96, BE-9000 Ghent, Belgium, phone: +32 9 221 8844; fax: +32 9 3324971, e-mail:
Folia Neuropathol. 2014;52(4):421-7. doi: 10.5114/fn.2014.47843.
Progressive supranuclear palsy (PSP) is a degenerative disease affecting mainly the brain stem, basal ganglia and cerebellum. Associated cerebrovascular lesions, mainly small cerebral bleeds, are frequently observed in some neurodegenerative diseases such as Alzheimer dementia and rare in others such as frontotemporal lobar degeneration. The present post-mortem study investigates the prevalence and distribution of small cerebral bleeds in PSP brains.
Nineteen brains of PSP patients were compared to 12 age-matched controls. The prevalence and distribution of mini-bleeds were investigated on a coronal section of a cerebral hemisphere at the level of the mamillary bodies and on a horizontal section through the pons and cerebellum. In addition, out of these series T2*-weighted gradient-echo 7.0-Tesla magnetic resonance imaging (MRI) of 3 coronal sections of a cerebral hemisphere and of a brain stem and cerebellum was performed in 14 PSP and 11 control brains.
Although the total number of mini-bleeds was the same on neuropathological examination of both groups, they prevailed around the dentate nucleus of the cerebellum (p = 0.05) and in the tegmentum pontis (p = 0.05) of the PSP brains. On MRI the small bleeds were also more frequent around the dentate nucleus of the cerebellum (p = 0.02) and in the pons (p = 0.04) of PSP brains.
In PSP brains, mini-bleeds only prevail in the regions affected by the neurodegenerative process, similarly to what happens in frontotemporal lobar degeneration. They should be considered as the result of increased angiogenesis and microglial activation, leading to associated disturbances of the blood-brain barrier in the most affected regions of PSP. They are not indicative of cerebrovascular disease.
进行性核上性麻痹(PSP)是一种主要影响脑干、基底神经节和小脑的退行性疾病。在一些神经退行性疾病(如阿尔茨海默病性痴呆)中,常可见相关的脑血管病变,主要是小脑出血,而在其他疾病(如额颞叶变性)中则较为罕见。本尸检研究调查了PSP脑中小脑出血的患病率和分布情况。
将19例PSP患者的脑与12例年龄匹配的对照者的脑进行比较。在乳头体水平的大脑半球冠状切面以及通过脑桥和小脑的水平切面上,研究微出血的患病率和分布情况。此外,在这些系列研究中,对14例PSP患者和11例对照者的脑进行了大脑半球3个冠状切面以及脑干和小脑的T2*加权梯度回波7.0特斯拉磁共振成像(MRI)检查。
尽管两组神经病理学检查中微出血的总数相同,但在PSP脑的小脑齿状核周围(p = 0.05)和脑桥被盖部(p = 0.05)微出血更为常见。在MRI上,PSP脑的小脑齿状核周围(p = 0.02)和脑桥(p = 0.04)的小出血也更为频繁。
在PSP脑中,微出血仅在受神经退行性病变影响的区域更为常见,这与额颞叶变性的情况类似。它们应被视为血管生成增加和小胶质细胞活化的结果,导致PSP最受影响区域的血脑屏障相关紊乱。它们并非脑血管疾病的指征。