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Hirayama disease

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Abstract

Purpose

Hirayama disease is an initially progressive disease caused by cervical neck flexion compressing the anterior horns of the lower cervical spinal cord. It is primarily seen in young males of Indian or Asian descent. With increasing dispersion of these populations this condition is increasingly being encountered internationally. This grand round reviews this rare but increasingly recognized condition.

Materials and methods

We present a classic case of a young Indian male with progressive hand and forearm weakness. We discuss the typical clinical presentation, appropriate investigations and management of this condition.

Results

Our patient presented with oblique amyotrophy and underwent a diagnostic flexion MRI scan which revealed anterior translation of the posterior dura with compression of the anterior horns of the lower cervical cord. He has been successfully treated in a cervical collar.

Conclusions

This case illustrates the typical presentation, diagnostic investigations and treatment of Hirayama syndrome. It is hoped that this review will alert clinicians of this condition and optimize the management of affected individuals.

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References

  1. Pradhan S, Gupta RK (1997) Magnetic resonance imaging in juvenile asymmetric segmental spinal muscular atrophy. J Neurol Sci 146(2):133–138

    Article  PubMed  CAS  Google Scholar 

  2. Hirayama K, Toyokura Y, Tsubaki T (1959) Juvenile muscular atrophy of unilateral upper extremity; a new clinical entity. Psychiatr Neurol Jpn 61:2190–2197

    Google Scholar 

  3. Hirayama K (2008) Juvenile muscular atrophy of unilateral upper extremity (Hirayama disease)—half-century progress and establishment since its discovery. Brain Nerve 60(1):17–29

    PubMed  Google Scholar 

  4. Hirayama K (2000) Juvenile muscular atrophy of distal upper extremity (Hirayama disease). Int Med 39(4):283–290

    Article  CAS  Google Scholar 

  5. 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(1):38–45

    Article  PubMed  Google Scholar 

  6. Hirayama K, Tokumaru Y (2000) Cervical dural sac and spinal cord in juvenile muscular atrophy of distal upper extremity. Neurology 54(10):1922–1926

    Article  PubMed  CAS  Google Scholar 

  7. Tokumaru Y, Hirayama K (1989) Anterior shift of posterior lower cervical dura mater in patients with juvenile muscular atrophy of unilateral upper extremity. Clin Neurol 29:1237–1243

    CAS  Google Scholar 

  8. Kano M (1998) Electrophysiology of Hirayama disease. Neurol Med 48:324–328

    Google Scholar 

  9. Kuwabara S, Nakajima M, Hattori T et al (1999) Electrophysiology of juvenile muscular atrophy of unilateral upper limb (Hirayama’s disease). Clin Neurol 39:508–512

    CAS  Google Scholar 

  10. Kijimi M, Nakajima M, Hattori T et al (1996) Electrophysiological investigation of cold paraparesis. Clin Neurol 36:1452

    Google Scholar 

  11. Suyama C, Suzuki T, Sugimoto Y et al (1994) Efficacy of short-latency somatosensory evoked potential (S-SEP) in cases of Hirayama disease. Igaku Kensa 43:1717–1723

    Google Scholar 

  12. Shizukawa H, Imai T, Kobayashi N et al (1994) Cervical flexion-induced changes of motor evoked potentials by transcranial magnetic stimulation in a patient with Hirayama disease: juvenile muscular atrophy of unilateral upper extremity. Clin Neurol 34:500–504

    CAS  Google Scholar 

  13. Saito M, Hirayam K, Narabayashi H et al (1978) Juvenile muscular atrophy localized to the hand and forearm: histochemical and ultrastructural studies of biopsied muscles in 10 cases. Clin Neurol 18:247–253

    Google Scholar 

  14. Yamazaki M, Hirayama K (1990) Queckenstedt test: pressure pattern analysis in spinal subarachnoid block. Clin Neurol 30:247–253

    CAS  Google Scholar 

  15. Hirayama K, Tomonaga M, Kitano K et al (1985) The first autopsy case of juvenile muscular atrophy of unilateral upper extremity. Neurol Med 22:85–88

    Google Scholar 

  16. Araki K, Ueda Y, Michinaka C et al (1989) An autopsy case of juvenile muscular atrophy of unilateral upper extremity (Hirayama’s disease). J Jpn Soc Int Med 78:674–675

    Article  CAS  Google Scholar 

  17. Kira J, Ochi H (2001) Juvenile muscular atrophy of the distal upper limb (Hirayama disease) associated with atopy. J Neurol Neurosurg Psychiatry 70(6):798–801

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  18. Elsheikh B, Kissel JT, Christoforidis G et al (2009) Juvenile muscular atrophy of the distal arm “Hirayama disease”. Muscle Nerve 40(2):206–212

    Article  PubMed  Google Scholar 

  19. Tokumaru Y, Hirayama K (1992) A cervical collar therapy for nonprogressive juvenile spinal muscular atrophy of the distal upper limb (Hirayama’s disease). Clin Neurol 32:1102–1106

    CAS  Google Scholar 

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Acknowledgements

Glynny Kieser for her editorial input.

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Authors and Affiliations

  1. Department of Orthopaedic Surgery and Musculoskeletal Medicine, Canterbury School of Medicine, Canterbury District Health Board, University of Otago, 2 Riccarton Avenue, Christchurch, 8011, New Zealand

    David C. Kieser

  2. Division of Neuroradiology, University of Oxford, Oxford, UK

    P. J. Cox

  3. Department of Radiology, Canterbury District Health Board, Christchurch, New Zealand

    S. C. J. Kieser

Authors
  1. David C. Kieser
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  2. P. J. Cox
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  3. S. C. J. Kieser
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Correspondence to David C. Kieser.

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None of the authors have any potential conflict of interest.

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Kieser, D.C., Cox, P.J. & Kieser, S.C.J. Hirayama disease. Eur Spine J 27, 1201–1206 (2018). https://doi.org/10.1007/s00586-018-5545-9

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  • DOI: https://doi.org/10.1007/s00586-018-5545-9

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