脊髓性进行性肌萎缩(以下简称SPMA)的概念、分类、临床表现以及遗传关系等方面,近年来有许多进展。现将各型概述如下: Aran—Duchenne型SPMA 本型为古典的SPMA,最特征的病理改变是脊髓前角细胞和前根的变性。前角细胞部分脱落消失,残存者亦萎缩或染色质溶解。亦可见神经细胞被噬现象。相应的脊髓前根萎缩,髓鞘脱失。以上改变以颈髓最突出。临床表现为成人缓慢起病,通常以两手小肌群 Spinal progressive muscular atrophy (hereinafter referred to as SPMA) concept, classification, clinical manifestations and genetic relationship, etc., in recent years, there have been many advances. The various types are now summarized as follows: Aran-Duchenne type SPMA This type is the classical SPMA, the most characteristic pathological changes of spinal cord anterior horn cells and anterior degeneration. Some anterior horn cell shedding disappeared, the remaining atrophy or chromatin dissolution. Nerve cells can also be seen phagocytosis phenomenon. The corresponding spinal cord atrophy, myelin loss. The above changes to the most prominent cervical cord. Clinical manifestations of adult onset of slow, usually small hands with both hands muscles