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Indian Journal of Neurosurgery 2014; 03(02): 125-127
DOI: 10.4103/2277-9167.138924
Case Report
Thieme Medical and Scientific Publishers Private Ltd.

Hirayama disease

Chanabasappa V. Chavadi
,
Gowtham Jasti
,
Madhav Hegde
1   Ambedkar Medical College, Bengaluru, Karnataka, India
,
Anand Venugopal
› Author Affiliations
Subject Editor:
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Publication History

Publication Date:
18 January 2017 (online)

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Abstract

Hirayama disease (HD) is a rare cause of cervical myelopathy often misdiagnosed as motor neuron disease and easily missed on routine magnetic resonance imaging (MRI), at time where detection is crucial to prevent irreversible cord injury. Believed to be a cervical flexion myelopathy, HD differs from motor neuron diseases because of its nonprogressive course and pathologic findings of chronic microcirculatory changes in the lower cervical cord. Dynamic forward displacement of dura in flexion causes asymmetric flattening of lower cervical cord. While dynamic contrast MRI is diagnostic, routine study has high predictive value. A case of HD, etiopathogenesis, imaging and treatment is summarized.

Key words

Cervical myelopathy - hand wasting - Hirayama disease - magnetic resonance imaging
 

  • References

  • 1 Hirayama K. Non-progressive juvenile spinal muscular atrophy of the distal upper limb [Hirayama’s disease]. In: De Jong JM. editor Handbook of Clinical Neurology. Vol. 15. Amsterdam, the Netherlands: Elsevier; 1991. p 107-120
    Google Scholar
  • 2 Chen CJ, Chen CM, Wu CL, Ro LS, Chen ST, Lee TH. Hirayama disease: MR diagnosis. AJNR Am J Neuroradiol 1998; 19: 365-368
    PubMedGoogle Scholar
  • 3 Kikuchi S, Tashiro K, Kitagawa K, Iwasaki Y, Abe H. A mechanism of juvenile muscular atrophy localized in the hand and forearm (Hirayama’s disease): Flexion myelopathy with tight dural canal in flexion. Clin Neurol (Tokyo) 1987; 27: 412-419
    PubMedGoogle Scholar
  • 4 Jakhere S, Wagh V. Hirayama’s disease: The importance of flexion magnetic resonance imaging. J Postgrad Med 2011; 57: 48-50
    CrossrefPubMedGoogle Scholar
  • 5 Chen CJ, Hsu HL, Tseng YC, Lyu RK, Chen CM, Huang YC. et al Hirayama flexion myelopathy: Neutral-position MR imaging findings - importance of loss of attachment. Radiology 2004; 231: 39-44
    CrossrefPubMedGoogle Scholar
  • 6 Gandhi D, Goyal M, Bourque PR, Jain R. Case 68: Hirayama disease. Radiology 2004; 230: 692-696
    CrossrefPubMedGoogle Scholar
  • 7 Mukai E, Matsuo T, Muto T, Takahashi A, Sobue I. Magnetic resonance imaging of juvenile-type distal and segmental muscular atrophy of the upper extremities. Rinsho Shinkeigaku 1987; 27: 99-107
    PubMedGoogle Scholar
  • 8 Chiba S, Yonekura K, Nonaka M, Imai T, Matumoto H, Wada T. Advanced Hirayama disease with successful improvement of activities of daily living by operative reconstruction. Intern Med 2004; 43: 79-81
    CrossrefPubMedGoogle Scholar