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Objective To approach the clinical and pathologic features of CSMA.Method To analyse retrospectively the clinical data of these 11 CSMA cases.Result The 11 patients were all chronic onset,mainly mainifesting of limb weakness,muscular atrophy and walking unsteadily;5 cases were with serum enzyme rising slightly.8 of the EMG showed neurogenic damage while the other 3 were normal.Muscle pathologic all showed inequality of size in muscle fibers,10 cases were with myofiber type grouping in different degrees,8 cases were with nuclear ingression myofibers,7 cases with little keratosic fibers and 6 cases were with target fibers.Conclusion The clinical feature of CSMA mainly manifesting of limb weakness,muscular atrophy and walking unsteadily.Pathological study shows neurogenic damage,including myofiber type grouping,nuclear ingression myofiber, little keratosic and target fibers etc.