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

29 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

Fingerprint

Spinal Cord Diseases
Neck
Monomelic amyotrophy
Kyphosis
Neurosurgery
Diagnostic Errors
Retrospective Studies
Therapeutics
Conservative Treatment

Keywords

  • Cervical flexion myelopathy
  • Dynamic MR imaging
  • Hirayama disease

ASJC Scopus subject areas

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

Cite this

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

Hirayama disease : Clinical article. / Lin, Muh Shi; Kung, Woon Man; Chiu, Wen Ta; Lyu, Rong Kuo; Chen, Chi Jen; Chen, Tzu Yung.

In: Journal of Neurosurgery: Spine, Vol. 12, No. 6, 06.2010, p. 629-634.

Research output: Contribution to journalArticle

Lin, MS, Kung, WM, Chiu, WT, Lyu, RK, Chen, CJ & Chen, TY 2010, 'Hirayama disease: Clinical article', Journal of Neurosurgery: Spine, vol. 12, no. 6, pp. 629-634. https://doi.org/10.3171/2009.12.SPINE09431
Lin, Muh Shi ; Kung, Woon Man ; Chiu, Wen Ta ; Lyu, Rong Kuo ; Chen, Chi Jen ; Chen, Tzu Yung. / Hirayama disease : Clinical article. In: Journal of Neurosurgery: Spine. 2010 ; Vol. 12, No. 6. pp. 629-634.
@article{06b818c4ce6f4d96b03ca4df7bd8b205,
title = "Hirayama disease: Clinical article",
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.",
keywords = "Cervical flexion myelopathy, Dynamic MR imaging, Hirayama disease",
author = "Lin, {Muh Shi} and Kung, {Woon Man} and Chiu, {Wen Ta} and Lyu, {Rong Kuo} and Chen, {Chi Jen} and Chen, {Tzu Yung}",
year = "2010",
month = "6",
doi = "10.3171/2009.12.SPINE09431",
language = "English",
volume = "12",
pages = "629--634",
journal = "Journal of Neurosurgery: Spine",
issn = "1547-5654",
publisher = "American Association of Neurological Surgeons",
number = "6",

}

TY - JOUR

T1 - Hirayama disease

T2 - Clinical article

AU - Lin, Muh Shi

AU - Kung, Woon Man

AU - Chiu, Wen Ta

AU - Lyu, Rong Kuo

AU - Chen, Chi Jen

AU - Chen, Tzu Yung

PY - 2010/6

Y1 - 2010/6

N2 - 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.

AB - 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.

KW - Cervical flexion myelopathy

KW - Dynamic MR imaging

KW - Hirayama disease

UR - http://www.scopus.com/inward/record.url?scp=77953932868&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=77953932868&partnerID=8YFLogxK

U2 - 10.3171/2009.12.SPINE09431

DO - 10.3171/2009.12.SPINE09431

M3 - Article

C2 - 20515348

AN - SCOPUS:77953932868

VL - 12

SP - 629

EP - 634

JO - Journal of Neurosurgery: Spine

JF - Journal of Neurosurgery: Spine

SN - 1547-5654

IS - 6

ER -