title: Myopathy associated with neuromyelitis optica spectrum disorders.
abstract: Neuromyelitis optica spectrum disorders (NMOSD) were generally thought to affect only central nervous system and spare peripheral aquaporin-4 (AQP4)-expressing organs. In recent years, however, increasing evidence has shown that skeletal muscle is involved in NMOSD. We provided a comprehensive review of the relevant literature and summarized the clinical and pathological characteristics of myopathy associated with NMOSD. NMOSD-associated myopathy seems to be characterized by mild muscle symptoms with prominent hyperCKemia and minimal changes on conventional pathological staining. Loss of AQP4 and deposition of IgG and activated complement products on sarcolemma of type II fibers are diagnostic features on immunohistochemical examinations. Creatine kinase leakage as a result of AQP4-IgG-induced, complement-mediated sarcolemmal injury may be a potential mechanism for hyperCKemia. Myopathy should be considered a component of NMOSD unified by AQP4-IgG seropositivity.
authors.affiliation: a Department of Neurology , Affiliated Hospital of Guizhou Medical University , Guiyang , China.
authors.forename: Dian
authors.initials: D
authors.lastname: He
authors.affiliation: a Department of Neurology , Affiliated Hospital of Guizhou Medical University , Guiyang , China.
authors.forename: Ya
authors.initials: Y
authors.lastname: Li
authors.affiliation: a Department of Neurology , Affiliated Hospital of Guizhou Medical University , Guiyang , China.
authors.forename: Qingqing
authors.initials: Q
authors.lastname: Dai
authors.affiliation: a Department of Neurology , Affiliated Hospital of Guizhou Medical University , Guiyang , China.
authors.forename: Yifan
authors.initials: Y
authors.lastname: Zhang
authors.affiliation: a Department of Neurology , Affiliated Hospital of Guizhou Medical University , Guiyang , China.
authors.forename: Zhu
authors.initials: Z
authors.lastname: Xu
authors.affiliation: a Department of Neurology , Affiliated Hospital of Guizhou Medical University , Guiyang , China.
authors.forename: Yuan
authors.initials: Y
authors.lastname: Li
authors.affiliation: a Department of Neurology , Affiliated Hospital of Guizhou Medical University , Guiyang , China.
authors.forename: Gang
authors.initials: G
authors.lastname: Cai
authors.affiliation: a Department of Neurology , Affiliated Hospital of Guizhou Medical University , Guiyang , China.
authors.forename: Lan
authors.initials: L
authors.lastname: Chu
mesh_terms: D006801:Humans; D009135:Muscular Diseases / Q000201:enzymology / Q000209:etiology* / Q000503:physiopathology; D009471:Neuromyelitis Optica / Q000150:complications*
keywords: AQP4-IgG; hyperCKemia; longitudinally extensive transverse myelitis; myopathy; neuromyelitis optica spectrum disorders; optic neuritis