新近Shuaib等报告,先天性肌病性中心空病(congenital myopathy centralcore disease)患者对全麻有潜在地致命性恶性高热反应的危险。作者报告的以肌无力为常见表现患者中的3例都是在恶性高热后才发现有本病。本病为常染色体显性遗传。临床特征:病情轻,呈非进行性,患者在婴幼儿期或儿童期就有肌张力低下及近端肌无力。诊断主要是根据肌活检见肌纤维中有“空壳”(呈中心性或偏心性,有时有多个病灶,甚至侵犯整个肌纤维)而确 Recently, Shuaib et al reported that patients with congenital myopathy centralcore disease are at risk for potentially fatal malignant hyperthermia in general anesthesia. Three of the patients reported with myasthenia gravis as common findings were found to have this disease after malignant hyperthermia. The disease is autosomal dominant. Clinical features: mild illness, non-progressive, patients with infantile or childhood low muscle tension and proximal muscle weakness. Diagnosis is mainly based on muscle biopsy muscle fibers in the “shell” (central or eccentric, and sometimes multiple lesions, and even violations of the entire muscle fiber) and indeed