• Atul Goel, Arjun Dhar, and Abhidha Shah.
• Department of Neurosurgery, King Edward Memorial Hospital, Parel, Mumbai, India. Electronic address: atulgoel62@hotmail.com.
• World Neurosurg. 2017 Mar 1; 99: 186-191.

ObjectiveTo analyze the role of multisegmental spinal instability in the pathogenesis of Hirayama disease.Material And MethodsFrom 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).ResultsThe 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.ConclusionsFrom the observations, it appears that atlantoaxial and subaxial spinal instability plays a major role in the pathogenesis of Hirayama disease.Copyright © 2016 Elsevier Inc. All rights reserved.

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