进行性皮质下胶质细胞增生症:不应忽视的神经变性痴呆

Progressive subcortical gliosis:neurodegenerative dementia should not be ignored

进行性皮质下胶质细胞增生症是临床少见的神经变性痴呆,病理学特点主要是额叶、颞叶等部位星形胶质细胞增生;临床表现为人格改变、进行性痴呆、构音障碍和肌阵挛等;头部MRI可见与神经胶质增生相关的T_2WI高信号。由于临床表现和辅助检查缺乏特异性,明确诊断主要依靠尸体解剖。神经影像学与特征性病理改变相结合必将为进行性皮质下胶质细胞增生症的生前诊断带来希望。

Progressive subcortical gliosis（PSG） is a rare neurodegenerative dementia,which is characterized of astrocytic proliferation in the frontal and temporal lobe.Clinical symptoms of PSG include personality changes,progressive dementia,dysarthria,myoclonia,etc.Brain MRI shows T2WI hyperintense,which is related to astrocytic hyperplasia.Due to poor specification both in clinical manifestation and examination,the definite diagnosis of PSG mainly depends on the result of autopsy.The combination of neuroimage and pathology will definitly give hope to build the diagonosis of PSG while patient is living.