title: Multilevel Spinal Stabilization as a Treatment for Hirayama Disease: Report of an Experience with Five Cases.
abstract: OBJECTIVE
  To analyze the role of multisegmental spinal instability in the pathogenesis of Hirayama disease.
  
  
  METHODS
  From June 2014 to January 2016, the authors managed 5 patients with Hirayama disease. The patients were diagnosed on the basis of classical described radiologic and clinical guidelines. All 5 patients were treated with multilevel cervical fixation that included fixation of the atlantoaxial joint in 4 patients by the adoption of the facetal fixation methods. No dural or bone decompression was performed. The follow-up ranged from 7 to 26 months (average 17.6 months).
  
  
  RESULTS
  The most remarkable feature was an immediate postoperative and progressive improvement in the symptoms of weakness, wasting, and deformity of hands in all patients. The other remarkable feature was an immediate postoperative reduction in extradural mass in all patients and its complete disappearance in 2 patients.
  
  
  CONCLUSIONS
  From the observations, it appears that atlantoaxial and subaxial spinal instability plays a major role in the pathogenesis of Hirayama disease.
authors.affiliation: Department of Neurosurgery, King Edward Memorial Hospital, Parel, Mumbai, India. Electronic address: atulgoel62@hotmail.com.
authors.forename: Atul
authors.initials: A
authors.lastname: Goel
authors.affiliation: Department of Neurosurgery, King Edward Memorial Hospital, Parel, Mumbai, India.
authors.forename: Arjun
authors.initials: A
authors.lastname: Dhar
authors.affiliation: Department of Neurosurgery, King Edward Memorial Hospital, Parel, Mumbai, India.
authors.forename: Abhidha
authors.initials: A
authors.lastname: Shah
mesh_terms: D000293:Adolescent; D000328:Adult; D001268:Atlanto-Axial Joint / Q000000981:diagnostic imaging / Q000601:surgery*; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007593:Joint Instability / Q000150:complications / Q000175:diagnosis* / Q000601:surgery*; D008297:Male; D013123:Spinal Fusion / Q000295:instrumentation / Q000379:methods*; D014897:Spinal Muscular Atrophies of Childhood / Q000175:diagnosis* / Q000209:etiology / Q000601:surgery*; D016896:Treatment Outcome; D055815:Young Adult
keywords: Atlantoaxial instability; Facetal fixation; Hirayama disease