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CC BY-NC-ND 4.0 · Journal of Health and Allied Sciences NU 2021; 11(01): 40-43
DOI: 10.1055/s-0040-1718237
Case Report

An Interesting Case of Bilateral Upper Limb Wasting: Hirayama Disease

Priscilla Rubavathy Eugin
1   Department of General Medicine, Sri Manakula Vinayagar Medical College and Hospital, Pondicherry, India
,
Manokaran Chinnusamy
1   Department of General Medicine, Sri Manakula Vinayagar Medical College and Hospital, Pondicherry, India
,
Sathiyanarayanan Janakiraman
1   Department of General Medicine, Sri Manakula Vinayagar Medical College and Hospital, Pondicherry, India
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Abstract

Hirayama disease is a rare neurologic disease and is characterized by insidious unilateral or bilateral muscular atrophy and weakness of the forearms and hands without sensory or pyramidal signs. Our patient presented with bilateral upper limb wasting, which is a rarer variant of motor neuron disease. The diagnosis of Hirayama disease is based on dynamic magnetic resonance imaging (MRI). This case displays how dynamic cervical spine MRI can pick up dynamic cord compression and contributes to zero in the diagnosis of Hirayama disease.

Keywords

Hirayama disease - dynamic MRI - motor neuron disease


Publikationsverlauf

Artikel online veröffentlicht:
16. Oktober 2020

© 2020. Nitte University (Deemed to be University). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

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  • References

  • 1 Hirayama K, Toyokura Y, Tsubaki T. Juvenile muscular atrophy of unilateral upper extremity: a new clinical entity. J Psychiatr Neurol. 1959; 61: 2190-2197
    Google Scholar
  • 2 Hirayama K, Tomonaga M, Kitano K, Yamada T, Kojima S, Arai K. Focal cervical poliopathy causing juvenile muscular atrophy of distal upper extremity: a pathological study. J Neurol Neurosurg Psychiatry 1987; 50 (03) 285-290
    CrossrefPubMedGoogle Scholar
  • 3 Pal PK, Atchayaram N, Goel G, Beulah E. Central motor conduction in brachial monomelic amyotrophy. Neurol India 2008; 56 (04) 438-443
    CrossrefPubMedGoogle Scholar
  • 4 Lewis D, Saxena A, Herwadkar A, Leach J. A confirmed case in the United Kingdom of Hirayama disease in a young white male presenting with hand weakness. World Neurosurg 2017; 105: 1039.e7-1039.e12
    CrossrefPubMedGoogle Scholar
  • 5 Pradhan S, Gupta RK. Magnetic resonance imaging in juvenile asymmetric segmental spinal muscular atrophy. J Neurol Sci 1997; 146 (02) 133-138
    CrossrefPubMedGoogle Scholar
  • 6 Hirayama K. Non-progressive juvenile spinal muscular atrophy of the distal upper limb (Hirayama's disease). In: De Jong JM. ed. Handbook of Clinical Neurology. Vol. 15. Amsterdam: Elsevier; 1991: 107-120
    Google Scholar
  • 7 Toma S, Shiozawa Z. Amyotrophic cervical myelopathy in adolescence. J Neurol Neurosurg Psychiatry 1995; 58 (01) 56-64
    CrossrefPubMedGoogle Scholar
  • 8 Tashiro K, Kikuchi S, Itoyama Y. et al. Nationwide survey of juvenile muscular atrophy of distal upper extremity (Hirayama disease) in Japan. Amyotroph Lateral Scler 2006; 7 (01) 38-45
    CrossrefPubMedGoogle Scholar
  • 9 Chen CJ, Chen CM, Wu CL, Ro LS, Chen ST, Lee TH. Hirayama disease: MR diagnosis. AJNR Am J Neuroradiol 1998; 19 (02) 365-368
    PubMedGoogle Scholar
  • 10 Tokumaru Y, Hirayama K. Cervical collar therapy for juvenile muscular atrophy of distal upper extremity (Hirayama disease): results from 38 cases [in Japanese]. Rinsho Shinkeigaku 2001; 41 (4-5) 173-178
    PubMedGoogle Scholar