Mitsuyama Y, Itoi K
No To Shinkei. 1976 Aug;28(8):761-7.
A case of a 44-year-old male with progressive subcortical gliosis was presented. The clinical manifestations included mental activity, difficulty in attention, euphoria and inability to work. The impairement of memory was not evident and his personality was fairly well preserved. Neurological examination showed no abnormality. The course was progressive without remission and the patient died with pneumonia approximately fifteen months after the onset of the disease. Laboratory tests revealed unremarkable except for slight degree of glucosuria and elevated F.B.S. Electroencephalography and carotid angiography were within normal. Neuropathological finding revealed that brain weighed 1200 g. and moderate degree of atrophy on frontal to parietotemporal lobes was evident. Occipital lobe was not involved. Microscopic examination revealed such changes as fibrillary gliosis in the cerebral white matter and grey matter in the spinal cord. The lesion in the cerebral white matter was predominent in the frontal lobe. Striatum and brain stem also showed mild degree of fibrillary gliosis. No senility related change such as senile plaque, granulovacuolar degeneration and Alzheimer's neurofibrillary tangle was found. The clinicopathologic findings in this case supported a diagnosis of progressive subcortical gliosis which was first described by Neumann in 1949.
报告一例44岁男性进行性皮质下胶质增生症。临床表现包括精神活动、注意力障碍、欣快及无法工作。记忆力损害不明显，人格基本保持完好。神经系统检查未见异常。病程呈进行性，无缓解，患者在疾病发作约15个月后死于肺炎。实验室检查除轻度糖尿及空腹血糖升高外无异常。脑电图及颈动脉血管造影正常。神经病理学检查发现脑重1200g，额叶至顶颞叶有中度萎缩，枕叶未受累。显微镜检查显示脑白质及脊髓灰质有纤维性胶质增生等改变。脑白质病变以额叶为主。纹状体及脑干也有轻度纤维性胶质增生。未发现老年斑、颗粒空泡变性及阿尔茨海默病神经原纤维缠结等与衰老相关的改变。该病例的临床病理表现支持进行性皮质下胶质增生症的诊断，该病由诺伊曼于1949年首次描述。
