Is there cervical spine muscle weakness in patients with Hirayama disease? A morphological study about cross-sectional areas of muscles on MRI



Patients with Hirayama disease (HD) present with a larger range of neck flexion and show signs of cervical spine instability. Cervical spine stability largely relies on cervical spine muscles. The purpose of this study was to compare the cross-sectional areas (CSAs) of cervical spine muscles between patients with HD and healthy controls, providing some insights into whether there is cervical spine muscle weakness and incongruence in HD patients.


In this retrospective study, cervical spine muscles CSAs of 44 HD patients, as well as that of 44 age- and sex-matched healthy counterparts, were measured on the T2-weighted axial MR images. The ratios of cervical spine muscles CSA to the corresponding vertebral body areas, defined as R-CSAs, and the flexor/extensor CSA ratios were computed and compared between two groups.


Compared with healthy counterparts, R-CSAs of total cervical spine muscles, total extensors, superficial extensors, and deep flexors were significantly lower in HD patients. HD patients also demonstrated a significantly greater superficial flexor/superficial extensor CSA ratio than the healthy counterparts, indicating a mismatch between superficial flexors CSA and superficial extensors CSA in HD patients.


In this pioneering study, HD patients had decreased size in most cervical spine muscles and a mismatch between CSAs of superficial flexor and that of superficial extensors. These results indicate generalized weakness and incongruence of cervical spine muscles, which may predispose cervical spine of HD patients to a less stable situation.

Graphic abstract

These slides can be retrieved under Electronic Supplementary Material.

This is a preview of subscription content, log in to check access.

Access options

Buy single article

Instant unlimited access to the full article PDF.

US$ 39.95

Price includes VAT for USA

Subscribe to journal

Immediate online access to all issues from 2019. Subscription will auto renew annually.

US$ 199

This is the net price. Taxes to be calculated in checkout.

Fig. 1



Body mass index


Cross-sectional area


Deep extensors


Deep flexors


Hirayama disease


Magnetic resonance imagine


Relative cross-sectional area


Region of interest


Superficial extensors


Superficial flexor


Total extensors


Total flexors


Total cervical spine muscles


Vertebral body area


  1. 1.

    Hirayama K, Tokumaru Y (2000) Cervical dural sac and spinal cord in juvenile muscular atrophy of distal upper extremity. Neurology 54:1922–1926

  2. 2.

    Tashiro K, Kikuchi S, Itoyama Y et al (2006) Nationwide survey of juvenile muscular atrophy of distal upper extremity (Hirayama disease) in Japan. Amyotroph Lateral Scler 7:38–45.

  3. 3.

    Watanabe K, Hasegawa K, Hirano T et al (2005) Anterior spinal decompression and fusion for cervical flexion myelopathy in young patients. J Neurosurg Spine 3:86–91.

  4. 4.

    Huang YL, Chen CJ (2011) Hirayama disease. Neuroimaging Clin N Am 21:939–950.

  5. 5.

    Lai V, Wong YC, Poon WL et al (2011) Forward shifting of posterior dural sac during flexion cervical magnetic resonance imaging in Hirayama disease: an initial study on normal subjects compared to patients with Hirayama disease. Eur J Radiol 80:724–728.

  6. 6.

    Goel A, Dhar A, Shah A (2017) Multilevel spinal stabilization as a treatment for Hirayama disease: report of an experience with five cases. World Neurosurg 99:186–191.

  7. 7.

    Yin B, Liu L, Geng DY (2011) Features of Hirayama disease on fully flexed position cervical MRI. J Int Med Res 39:222–228.

  8. 8.

    Xu X, Han H, Gao H et al (2011) The increased range of cervical flexed motion detected by radiographs in Hirayama disease. Eur J Radiol 78:82–86.

  9. 9.

    Wang H, Sun C, Yang S et al (2018) Dynamic cervical radiographs in patients with Hirayama disease: an unneglectable factor on the choice of surgery options. World Neurosurg 114:e433–e440.

  10. 10.

    Liu X, Sun Y (2013) The correlation analysis of the cervical spine alignment flexion ROM of adjacent segments with the spinal cord atrophy in Hirayama disease. Chin J Spine Spinal Cord 23:514–519

  11. 11.

    Panjabi MM, Cholewicki J, Nibu K et al (1998) Critical load of the human cervical spine: an in vitro experimental study. Clin Biomech (Bristol, Avon) 13:11–17

  12. 12.

    Thakar S, Mohan D, Furtado SV et al (2014) Paraspinal muscle morphometry in cervical spondylotic myelopathy and its implications in clinicoradiological outcomes following central corpectomy: clinical article. J Neurosurg Spine 21:223–230.

  13. 13.

    Jones EJ, Bishop PA, Woods AK, Green JM (2008) Cross-sectional area and muscular strength: a brief review. Sport Med 38:987–994.

  14. 14.

    Blazevich AJ, Coleman DR, Horne S, Cannavan D (2009) Anatomical predictors of maximum isometric and concentric knee extensor moment. Eur J Appl Physiol 105:869–878.

  15. 15.

    Okada E, Matsumoto M, Ichihara D et al (2011) Cross-sectional area of posterior extensor muscles of the cervical spine in asymptomatic subjects: a 10-year longitudinal magnetic resonance imaging study. Eur Spine J 20:1567–1573.

  16. 16.

    Thakar S, Kurudi Siddappa A, Aryan S et al (2017) Does the mesodermal derangement in Chiari Type I malformation extend to the cervical spine? Evidence from an analytical morphometric study on cervical paraspinal muscles. J Neurosurg Spine 27:421–427.

  17. 17.

    Elliott JM, Jull GA, Noteboom JT et al (2007) Magnetic resonance imaging study of cross-sectional area of the cervical extensor musculature in an asymptomatic cohort. Clin Anat 20:35–40.

  18. 18.

    Takeuchi K, Yokoyama T, Aburakawa S et al (2005) Axial symptoms after cervical laminoplasty with C3 laminectomy compared with conventional C3–C7 laminoplasty: a modified laminoplasty preserving the semispinalis cervicis inserted into axis. Spine (Phila Pa 1976) 30:2544–2549

  19. 19.

    Hirayama K (2008) Juvenile muscular atrophy of unilateral upper extremity (Hirayama disease)–half-century progress and establishment since its discovery. Brain Nerve 60:17–29

  20. 20.

    Garfin SR, Eismont FJ, Bell GR et al (2018) Rothman-Simeone and Herkowitz’s, the spine, 7th edn. Elsevier, Philadelphia

  21. 21.

    Ozaki H, Kubota A, Natsume T et al (2018) Effects of drop sets with resistance training on increases in muscle CSA, strength, and endurance: a pilot study. J Sport Sci 36:691–696.

  22. 22.

    Enix DE, Scali F, Pontell ME (2014) The cervical myodural bridge, a review of literature and clinical implications. J Can Chiropr Assoc 58:184–192

  23. 23.

    Yoon SY, Moon HI, Lee SC et al (2018) Association between cervical lordotic curvature and cervical muscle cross-sectional area in patients with loss of cervical lordosis. Clin Anat 31:710–715.

  24. 24.

    Cheng CH, Lin KH, Wang JL (2008) Co-contraction of cervical muscles during sagittal and coronal neck motions at different movement speeds. Eur J Appl Physiol 103:647–654.

  25. 25.

    Cheng CH, Cheng HY, Chen CP et al (2014) Altered Co-contraction of cervical muscles in young adults with chronic neck pain during voluntary neck motions. J Phys Ther Sci 26:587–590.

  26. 26.

    Lin MS, Kung WM, Chiu WT et al (2010) Hirayama disease. J Neurosurg Spine 12:629–634.

  27. 27.

    Lu F, Wang H, Jiang J et al (2013) Efficacy of anterior cervical decompression and fusion procedures for monomelic amyotrophy treatment: a prospective randomized controlled trial: clinical article. J Neurosurg Spine 19:412–419.

  28. 28.

    Tokumaru Y, Hirayama K (2001) Cervical collar therapy for juvenile muscular atrophy of distal upper extremity (Hirayama disease): results from 38 cases. Rinsho Shinkeigaku 41:173–178

  29. 29.

    Fu Y, Qin W, Sun QL, Fan DS (2016) Investigation of the compliance of cervical collar therapy in 73 patients with Hirayama disease. Zhonghua Yi Xue Za Zhi 96:3485–3488.

  30. 30.

    Borisut S, Vongsirinavarat M, Vachalathiti R, Sakulsriprasert P (2013) Effects of strength and endurance training of superficial and deep neck muscles on muscle activities and pain levels of females with chronic neck pain. J Phys Ther Sci 25:1157–1162.

  31. 31.

    Gross AR, Paquin JP, Dupont G et al (2016) Exercises for mechanical neck disorders: a cochrane review update. Man Ther 24:25–45.

Download references


We thank the support from colleagues in our department. Our special thanks go to Dr. Eric Rebich, from Harborview Medical Center, University of Washington, Seattle, USA, for providing language help. We also appreciate the grant from the Startup Fund for scientific research, Fujian Medical University (2018QH1072).


This study was supported by a grant from Startup Fund for scientific research, Fujian Medical University (2018QH1072).

Author information

Correspondence to Jianhua Lin.

Ethics declarations

Conflict of interest

The authors declare that they have no conflict of interest.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Electronic supplementary material

Below is the link to the electronic supplementary material.

Supplementary file1 (PPTX 141 kb)

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Li, Z., Zhang, W., Wu, W. et al. Is there cervical spine muscle weakness in patients with Hirayama disease? A morphological study about cross-sectional areas of muscles on MRI. Eur Spine J (2020).

Download citation


  • Cervical spine
  • Muscle
  • Cross-sectional area
  • MRI
  • Hirayama disease