Journal of Neurology

, Volume 258, Issue 11, pp 2078–2079 | Cite as

Waterskier’s Hirayama syndrome

  • Peter BedeEmail author
  • Arun L. W. Bokde
  • Susan C. Byrne
  • Marwa Elamin
  • Ronan J. Walsh
  • Orla Hardiman
Letter to the Editors

Dear Sirs,

Hirayama disease is a segmental inferior cervical myelopathy. Presentation is usually in young males with unilateral or asymmetrical upper limb weakness and atrophy. The clinical features can be similar to the initial presentation of amyotrophic lateral sclerosis, but Hirayama syndrome has a benign course and is typically limited to distal muscles of the upper limbs.

The typical teenage onset of the syndrome corresponds with juvenile growth spurt. The disproportionate growth of the vertebral column compared to the spinal cord resulting in the relative shortening of the dorsal roots and the loss of attachment between the posterior dural sac and the subjacent lamina are considered to be contributing factors [1].

Several reports document antecedent events including repetitive movements, competitive sport or strenuous physical work. Jeannet et al. [2] documented the case of a 10-year-old girl who had a nocturnal rhythmic movement disorder, leading to prolonged repetitive neck...


Amyotrophic Lateral Sclerosis Multifocal Motor Neuropathy Motor Unit Activation Potential Neck Flexion Sensory Nerve Action Potential 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Conflict of interest



  1. 1.
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    Jeannet PY, Kuntzer T, Deonna T, Roulet-Perez E (2005) Hirayama disease associated with a severe rhythmic movement disorder involving neck flexions. Neurology 64:1478–1479PubMedCrossRefGoogle Scholar
  3. 3.
    Huang YH, Ro LS, Chang HS et al (2008) A clinical study of Hirayama disease in Taiwan. Muscle Nerve 37:576–582PubMedCrossRefGoogle Scholar
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    Chen CJ, Hsu HL, Tseng YC (2004) Hirayama flexion myelopathy: neutral-position MR imaging findings—importance of loss of attachment. Radiology 231:39–44PubMedCrossRefGoogle Scholar
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    Sonwalkar HA, Shah RS, Khan FK, Gupta AK, Bodhey NK, Vottath S, Purkayastha S (2008) Imaging features in Hirayama disease. Neurol India 56:22–26PubMedCrossRefGoogle Scholar
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    Baba Y, Nakajima M, Utsunomiya H et al (2004) Magnetic resonance imaging of thoracic epidural venous dilation in Hirayama disease. Neurology 62:1426–1428PubMedGoogle Scholar

Copyright information

© Springer-Verlag 2011

Authors and Affiliations

  • Peter Bede
    • 1
    Email author
  • Arun L. W. Bokde
    • 1
  • Susan C. Byrne
    • 1
  • Marwa Elamin
    • 1
  • Ronan J. Walsh
    • 2
  • Orla Hardiman
    • 1
  1. 1.Trinity College DublinDublinIreland
  2. 2.Hermitage Medical ClinicDublinIreland

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