Neurological Sciences

, Volume 36, Issue 9, pp 1695–1696 | Cite as

Hirayama disease: the importance of adequate flexion MRI for diagnosis

  • Marinos KontzialisEmail author
  • Noushin Yahyavi-Firouz-Abadi
  • Carlos A. Zamora
Letter to the Editor

Dear Editor,

We read with great interest the recent correspondence by Cortese et al. [1], who described the importance of an early diagnosis of Hirayama disease (HD) in a patient who underwent conservative therapy. Herein, we stress the critical role of adequate flexion during dynamic MRI in the diagnosis of HD based on our recent experience.

HD is a benign self-limiting disease that causes distal upper limb weakness and atrophy, which progresses over 2–4 years, followed by spontaneous arrest usually within 5 years. HD is thought to occur secondary to spinal cord compression by the posterior dural sac during neck flexion leading to ischemic damage to the anterior horns. The dynamic anterior shift of the dural sac is considered indicative of early progressive disease [2]. Imaging findings on neutral position MRI that should alert the physician include loss of normal cervical spine lordosis, loss of attachment between the posterior dural sac and subjacent lamina, lower cervical cord...


Hirayama disease Flexion MRI Myelopathy Cervical 


Conflict of interest

None declared.


  1. 1.
    Cortese R, Gerevini S, Dicuonzo F, Zoccolella S, Simone IL (2015) Hirayama disease: the importance of an early diagnosis. Neurol Sci. doi: 10.1007/s10072-015-2080-x Google Scholar
  2. 2.
    Huang YL, Chen CJ (2011) Hirayama disease. Neuroimaging Clin N Am 21(4):939–950. doi: 10.1016/j.nic.2011.07.009 CrossRefPubMedGoogle Scholar
  3. 3.
    Chen CJ, Hsu HL, Tseng YC, Lyu RK, Chen CM, Huang YC, Wang LJ, Wong YC, See LC (2004) Hirayama flexion myelopathy: neutral-position MR imaging findings—importance of loss of attachment. Radiology 231(1):39–44. doi: 10.1148/radiol.2311030004 CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag Italia 2015

Authors and Affiliations

  • Marinos Kontzialis
    • 1
    Email author
  • Noushin Yahyavi-Firouz-Abadi
    • 1
  • Carlos A. Zamora
    • 1
  1. 1.Division of Neuroradiology, The Russell H. Morgan Department of Radiology and Radiological ScienceJohns Hopkins University School of MedicineBaltimoreUSA

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