Hirayama’s Disease – A Rare Case Report with Review of Literature

 

What to Learn from this Article?

Presentation and Diagnosis of Focal Amyotrohic (Hirayama’s Disease).


Case Report |  Volume 3 | Issue 3 | JOCR July-Sep 2013 | Page 11-14 | Narayana Gowda BS, Mohan Kumar J, Basim PK.


Authors: Narayana Gowda BS[1], Mohan Kumar J[1], Basim PK[1].

[1]PES Medical College, Kuppam, Chittore, Andra Pradesh.

 Address of Correspondence:

Dr Narayana Gowda BS: Asst Prof, PES Medical College, Kuppam, Chittore, Andra Pradesh. Email: drnarayan999@yahoo.com


Abstract

Introduction: Hirayama’s disease is a rare benign disorder, also referred to as monomelic amyotrophy (MMA), Juvenile non progressive amyotrophy, Sobue disease. It is a focal, lower motor neuron type of disease. Mainly young males in their second and third decades of age are most commonly affected. It is seen most commonly in Asian countries like India and Japan. In majority of people cause of this disease is unknown. MRI of cervical spine in flexion will reveal the cardinal features of Hirayama disease.

Case Report: A 22 year gentleman came with a history of insidious onset of weakness in both the hands begenning with left side followed by right of 4 years duration. On examination he had clawing of both hands with wasting of forearm muscles. Lower limbs had no abnormality with normal deep tendon reflexes.  MRI showed thinning of cord from C4 to C7 level suggestive of cord atrophy. Based on these features a diagnosis of focal amyotrophy was made. A cervical collar was prescribed and patient is under regular follow up.

Conclusion: Hirayama disease is a rare self-limiting disease. Early diagnosis is necessary as the use of a simple cervical collar which will prevent neck flexion, has been shown to stop the progression.

Keywords: Hirayama’s disease, monomelic amyotrophy, Juvenile non-progressive amyotrophy, Sobue disease.


 

How to Cite This Article: Narayana Gowda BS, Mohan Kumar J, Basim PK. Hirayama’s Disease – A Rare Case Report with Review of Literature. Journal of Orthopaedic Case Reports 2013 July-Sep ;3(3): 11-14. Available from: http://www.jocr.co.in/wp/2013/07/11/2250-0685-107-fulltext/


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