Hirayama disease: Clinical article

Muh Shi Lin, Woon Man Kung, Wen Ta Chiu, Rong Kuo Lyu, Chi Jen Chen, Tzu Yung Chen

Research output: Contribution to journalArticle

30 Citations (Scopus)

Abstract

Object. Controversy exists over the choice of surgical candidates and prognosis of Hirayama disease. The purpose of this study was to examine the outcomes of patients with cervical flexion myelopathy who received surgical treatment. Methods. A retrospective study was conducted. From May 2002 through December 2006, 6 young patients with cervical flexion myelopathy were seen in the Department of Neurosurgery at Chang Gung Memorial Hospital. The neurological and radiological findings in all 6 patients met the criteria for Hirayama disease. All patients had evidence of a tight dural canal or forward migration of the posterior wall of the dural canal in dynamic MR imaging studies. Five patients were treated with surgical decompressive procedures (4 anterior and 1 posterior) and 1 patient received conservative treatment. Duration of follow-up ranged from 13 months to 4 years. Results. Motor function improved in 3 of 5 surgically treated patients and sensory function improved in 2. Neurological symptoms were unchanged in the conservatively treated patient. During follow-up MR imaging in the surgical group, anterior effacement during neck flexion was noted in 1 patient treated with a posterior approach. Conclusions. Hirayama disease is so rare that it is easily misdiagnosed. Diagnosis is achieved via clinical presentation, neurophysiological examination, and neuroradiological imaging studies (dynamic MR imaging). The anterior decompressive approach may be better for patients showing anterior effacement and severe cervical kyphosis during neck flexion in MR imaging.

Original languageEnglish
Pages (from-to)629-634
Number of pages6
JournalJournal of Neurosurgery: Spine
Volume12
Issue number6
DOIs
Publication statusPublished - Jun 2010

Keywords

  • Cervical flexion myelopathy
  • Dynamic MR imaging
  • Hirayama disease

ASJC Scopus subject areas

  • Clinical Neurology
  • Surgery
  • Neurology
  • Medicine(all)

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    Lin, M. S., Kung, W. M., Chiu, W. T., Lyu, R. K., Chen, C. J., & Chen, T. Y. (2010). Hirayama disease: Clinical article. Journal of Neurosurgery: Spine, 12(6), 629-634. https://doi.org/10.3171/2009.12.SPINE09431