Vermersch P, Daems-Monpeurt C, Parent M, Pruvo J P, Delacourte A, Petit H
Clinique Neurologique, Hôpital B, CHRU de Lille.
Rev Neurol (Paris). 1994;150(5):354-8.
A 36 year-old patient presented with a dementia of frontal type, gait disturbances, incontinence and a pseudo-bulbar palsy, which caused death at age 40. Brain biopsy of the frontal lobe showed an extensive deep subcortical gliosis. A high level of GFAP was detected by immunoblotting in the biopsy. Clinical and neuropathological observations are similar to cases described as Neumann Progressive Subcortical Gliosis. Single Photon Emission Computer Tomography showed a bilateral frontotemporal hypoperfusion, and Magnetic Resonance Imaging large periventricular and subcortical hyperintensities in both hemispheres, the brainstem and the cerebellum. The hyperintensities on T2-weighted MR images might be related to the intense gliosis. The contribution of such imaging data to diagnosis must be confirmed by other clinico-pathological cases.
一名36岁患者出现额型痴呆、步态障碍、尿失禁和假性延髓麻痹，于40岁时死亡。额叶脑活检显示广泛的深部皮质下胶质增生。活检中通过免疫印迹检测到高水平的胶质纤维酸性蛋白（GFAP）。临床和神经病理学观察结果与描述为诺伊曼进行性皮质下胶质增生的病例相似。单光子发射计算机断层扫描显示双侧额颞叶灌注不足，磁共振成像显示双侧大脑半球、脑干和小脑有大量脑室周围和皮质下高信号。T2加权磁共振图像上的高信号可能与强烈的胶质增生有关。此类影像学数据对诊断的贡献必须通过其他临床病理病例来证实。
