COURSE AND SYMPTOMS OF PROGRESSIVE INFANTILE MUSCULAR ATROPHY

摘要: PROGRESSIVE infantile muscular atrophy was first described by Werdnig1and Hoffmann2about fifty years ago. This disorder is defined as a hereditary familial degeneration of the motor cells in anterior horns spinal cord causing progressive and paralysis skeletal muscles. The begins muscles back proximal limbs spreads gradually to whole body. Life threatened when nerve supplying respiratory are affected, particularly this involvement followed infection tract. Up time report, approximately 200 cases, occurring about 120 families, have been reported. However, reader will still find best description disease Hoffmann's papers. Many later single observations short series cases contributed our knowledge its relation