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Objective SBMA is traditionally considered as a motoneuron disease.But elevation of serum creatine kinase (CK) has been reported in SBMA patients in different cohorts.Since many neuromuscular disorders can present with elevated serum CK.it will cause a diagnostic dilemma and pose a speculation that SBMA is not a pure motoneuron disease.We will study 13 patients with SBMA in Chinese and analyze the mechanism of hyperCKemia.