Kato Hiroko, Yoshida Mari, Ando Tetsuo, Sugiura Makoto, Hashizume Yoshio
Department of Neurology, Anjo Kosei Hospital.
Rinsho Shinkeigaku. 2009 Jun;49(6):348-53. doi: 10.5692/clinicalneurol.49.348.
A 48-years-old man presented with slowly progressive bradykinesia, personality change and rapidly progressive left hemiparesis. On admission, he presented dementia, poor judgment, left hemiparesis. MRI revealed a widespread high intensity area in right hemisphere and MRA was almost normal. Serological tests of serum and CSF demonstrated high titers of antibodies to Treponema pallidum. He was treated for syphilis with daily penicillin injections without improvement. He died of sepsis eight months after admission. At autopsy, the brain weighed 1,100 g and the right cerebral hemisphere was atrophic, especially in frontal base, temporal, parietal, angular, and posterior regions covered by thickened, fibrotic leptomeninges. Microscopically, chronic meningoencephalitis was observed. Severe neuronal loss with gliosis was seen in the right cerebral cortices. Scattered rod-shaped microglia and inflammatory cell infiltration were visible in the cerebral parenchyma. The dorsal column of the spinal cord was not involved and meningovascular syphilis was unclear. The distribution of the encephalitic lesions was well correlated with the clinical and neuroradiological findings. This was a rare autopsy case presenting Lissauer's general paresis, clinically manifesting as rapidly progressive stroke-like episode.
一名48岁男性,表现为缓慢进展的运动迟缓、人格改变以及快速进展的左侧偏瘫。入院时,他存在痴呆、判断力差和左侧偏瘫。MRI显示右半球广泛的高强度区域,MRA基本正常。血清和脑脊液的血清学检测显示梅毒螺旋体抗体滴度高。他接受了每日青霉素注射治疗梅毒,但病情无改善。入院八个月后死于败血症。尸检时,脑重1100克,右侧大脑半球萎缩,尤其是额叶底部、颞叶、顶叶、角回和后部区域,被增厚、纤维化的软脑膜覆盖。显微镜下,观察到慢性脑膜脑炎。右侧大脑皮质可见严重的神经元丢失伴胶质增生。脑实质内可见散在的杆状小胶质细胞和炎性细胞浸润。脊髓后索未受累,脑膜血管梅毒情况不明。脑炎病变的分布与临床和神经放射学表现密切相关。这是一例罕见的尸检病例,表现为李绍尔全身麻痹,临床上表现为快速进展的类似中风发作。