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亚急性海绵状脑病是一种由慢病毒非常规因子感染引起的罕见散发的中枢神经系统疾病。其病理为大脑皮层、基底节、小脑和脊髓等部位呈海绵状变性,神经细胞脱落坏死、胶质细胞重度增生和细孔样空泡形成。临床典型表现为亚急性进行性痴呆,伴有锥体系和/或锥体外系症状和肌阵挛。脑电图对该病的诊断有较高的特异性,但确诊仍依据病理检查。目前该病尚无特效疗法。患者的血、脑脊液和脑组织都是潜在的传染源,应预防其传播感染。
Subacute Spongiform Encephalopathy is a rare central nervous system disorder caused by infection with a lentiviral non-regulatable factor. The pathology of the cerebral cortex, basal ganglia, cerebellum and spinal cord and other parts of sponge-like degeneration, nerve cell loss of necrosis, glial cell hyperplasia and pore-like vacuoles formation. Typical clinical manifestations of subacute progressive dementia, with cones and / or extrapyramidal symptoms and myoclonus. EEG diagnosis of the disease has a high specificity, but the diagnosis is still based on pathological examination. Currently there is no cure for the disease. The patient’s blood, cerebrospinal fluid and brain tissue are potential sources of infection and should prevent their spread of infection.