title: Correlations of clinical, neuroimaging, and electrophysiological features in Hirayama disease.
abstract: Hirayama disease (HD) is characterized by development of asymmetric forearm muscle atrophy during adolescence with or without focal cervical spinal cord atrophy. The purpose of this study is to assess the correlation of clinical symptoms, disease progression, and electrophysiological findings with cervical spine magnetic resonance imaging (MRI) findings.The medical records, cervical spine MRIs, and electrophysiological findings of 44 HD patients were retrospectively reviewed and analyzed.Denervation changes in any single C5 to C7 root-innervated muscle (deltoid, biceps, triceps, or extensor digitorum communis) occurred more frequently in the 25 patients with cord atrophy than the 19 patients without cord atrophy (88% vs 53%, P = 0.02). Onset age, duration of disease progression, neurological examinations, nerve conduction study, and electromyographic findings from individual muscles were similar between patient groups.Compared with HD patients without cord atrophy, HD patients with cord atrophy experience a more severe denervation change in C5 to C7 root-innervated muscles.
authors.affiliation: Section of Neuromuscular Disease, Department of Neurology, Chang Gung Memorial Hospital-Linkou Medical Center, Chang Gung University College of Medicine, Taoyuan, Taiwan.
authors.forename: Ming-Feng
authors.initials: MF
authors.lastname: Liao
authors.forename: Hong-Shiu
authors.initials: HS
authors.lastname: Chang
authors.forename: Kuo-Hsuan
authors.initials: KH
authors.lastname: Chang
authors.forename: Long-Sun
authors.initials: LS
authors.lastname: Ro
authors.forename: Chun-Che
authors.initials: CC
authors.lastname: Chu
authors.forename: Hung-Chou
authors.initials: HC
authors.lastname: Kuo
authors.forename: Rong-Kuo
authors.initials: RK
authors.lastname: Lyu
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D002574:Cervical Vertebrae / Q000503:physiopathology*; D003714:Denervation; D018450:Disease Progression; D004576:Electromyography; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009431:Neural Conduction; D009460:Neurologic Examination; D012189:Retrospective Studies; D014897:Spinal Muscular Atrophies of Childhood / Q000175:diagnosis* / Q000503:physiopathology*; D055815:Young Adult