Rare case of Hirayama’s disease

Authors

  • Rohan Bharat Gala Department of Orthopaedics, D.Y. Patil University School of Medicine, Nerul, Navi Mumbai, Maharashtra
  • Sudhanshu Rakesh Vyas Department of Orthopaedics, D.Y. Patil University School of Medicine, Nerul, Navi Mumbai, Maharashtra
  • Amit B. Dhond Department of Orthopaedics, D.Y. Patil University School of Medicine, Nerul, Navi Mumbai, Maharashtra
  • Akshay Hemant Shah Department of Orthopaedics, D.Y. Patil University School of Medicine, Nerul, Navi Mumbai, Maharashtra
  • Ravindra G. Khedekar Department of Orthopaedics, D.Y. Patil University School of Medicine, Nerul, Navi Mumbai, Maharashtra
  • Sunil H. Shetty Department of Orthopaedics, D.Y. Patil University School of Medicine, Nerul, Navi Mumbai, Maharashtra

Keywords:

Hirayama’s disease, Monomelic amyotrophy, Juvenile Muscular atrophy of distal upper extremity

Abstract

Hirayama’s disease is a rare benign neurological disorder also known as monomelic amyotrophy, Sobue disease,Juvenile Muscular Atrophy of Distal Upper Extremity (JMADUE). It mainly affects young males in their second or third decades and is most commonly seen in Asian countries like Japan, Malaysia and India. In majority of the cases the cause of the disease is unknown. An 18 year male came with weakness in his right hand and forearm since 1 year. Examination revealed weakness and wasting of muscles of forearm and hand without lower limb involvement and normal deep tendon reflexes. MRI showed focal short segment hyperintense signal in the ventral and right lateral aspect of the cervical cord at C5-C6 level with the involved segment measuring 4x3mm in size. Based on clinical and radiological features a diagnosis of focal amyotrophy was made. Patient is given a cervical collar to prevent flexion at the neck and physiotherapy in the form of hand and forearm exercises were started. Regular follow up of the patient once every 2 months is being done. Hirayama’s disease is a rare, benign, self-limiting neurological disorder. Early diagnosis and management by preventing cervical flexion with the help of a cervical collar has shown to halt the progression of the disease.

References

Gourie-Devi M. Monomelic amyotrophy of upper or lower limbs. In: Wisen AA, Shaw PJ, eds. Handbook of Clinical Neurology. 1st ed. US: Elsevier, BV; 2007:207-227.

Hirayama K, Tokumaru Y. Cervical dural sac and spinal cord in juvenile muscular atrophy of distal upper extremity. Neurology. 2000;54:1922-6.

Hirayama K. Non-progressive juvenile spinal muscular atrophy of distal upper limb (Hirayama’s disease). In: De Jong JMBV, eds. Handbook of Clinical Neurology. 15th ed. Amsterdam, the Netherlands: Elsevier;1991:107-120.

Hirayama K, Toyokura Y, Tsubaki T. Juvenile muscular atrophy of unilateral upper extremity: a new clinical entity. Psychiatr Neurol Jpn. 1959;61:2190-7.

Nascimento OJ, Freitas MR. Non progressive juvenile spinal muscular atrophy of the distal upper limb (Hirayama’s disease): a clinical variant of the benign monomelic amyotrophy. Arq Neuropsiquiatr. 2000;58:814-9.

Gandhi D, Goyal M, Bourque PR, Jain R. Case 68: Hirayama’s disease. Radiology. 2004;230:692-6.

Kikuchi S, Tashiro K. Juvenile muscular atrophy of distal upper extremity (Hirayama disease). In: Jones HR Jr, De Vivo DC, Darras BT, eds. Disorders of Infancy, Childhood and Adolescence-A Clinician’s Approach. 29th ed. UK: Butterworth-Heinemann; 2003:167-181.

Chan YW, Kay R, Schwartz MS. Juvenile distal spinal muscular atrophy of upper extremities in Chinese males: a single fiber electromyographic study of arms and legs. J Neurol Neurosurg Psychiatry. 1991;54:165-76.

Peiris JB, Seneviratne KN, Wickremasinghe HR, Gunatilake SB, Gamage R. Nonfamilial juvenile distal spinal muscular atrophy of upper extremity. J Neurol Neurosurg Psychiatry. 1989;52:314-9.

Ochi H, Murai H, Oseogawa M, Minohara M, Inaba S, Kira J. Juvenile muscular atrophy of distal upper extremity associated with airway allergy: two cases successfully treated by plasma exchange. J Neurol Sci. 2003;206:109-14.

Chiba S, Yonekura K, Nonaka M, Imai J, Matumoto H, Wada T, et al. Advanced Hirayama disease with successful improvements in activities of daily living by operative reconstruction. Intern Med. 2004;43:79-81.

Mukai E, Matsuo T, Muto T, Takahashi A, Sobue I. Magnetic resonance imaging of juvenile type distal and segmental muscular atrophy of upper extremities. Clin Neurol (Tokyo). 1987;27:99-107.

Bland JH. Basic anatomy. In: Bland JH, eds. Disorders of the Cervical Spine: Diagnosis and Medical Management. 2nd ed. Philadelphia, Pa: Saunders; 1994: 41-70.

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Published

2017-01-07

How to Cite

Gala, R. B., Vyas, S. R., Dhond, A. B., Shah, A. H., Khedekar, R. G., & Shetty, S. H. (2017). Rare case of Hirayama’s disease. International Journal of Research in Medical Sciences, 3(3), 767–770. Retrieved from https://www.msjonline.org/index.php/ijrms/article/view/1372

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Section

Case Reports