Department of Medicine, Krishna Institute of Medical Sciences University, Karad, Maharashtra, India
Abstract
Hirayama's disease is a rare clinical variant of amyotrophic lateral sclerosis where distal muscles are involved more compared to proximal muscles and vice-versa occurs only in 10% cases and so it is differentiated from O'Sullivan McLeod syndrome which involves only small muscles of single limb. Here, we present a case of Hirayama's disease where disease achieved a plateau after 3 years with no further progression. His electrophysiological studies, and clinical picture, and magnetic resonance imaging findings were consistent with a diagnosis of Hirayama's disease.
Orsini M, Freitas MR, Catbarino A, Mello MP, Nascimento OJ. Upper limb proximal form of benign monomelic amyotrophy: On purpose of 2 cases. Rev Bras Neurol 2008;44:13-7.
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. 107-20.
Chan YW, Kay R, Schwartz MS. Juvenile distal spinal muscular atrophy of upper extremities in Chinese males: A single fibre electromyographic study of arms and legs. J Neurol Neurosurg Psychiatry 1991;54:165-6.
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.
Kikuchi S, Tashiro K. Juvenile muscular atrophy of distal upper extremity (Hirayama disease). In Jones HR Jr, De Vivo DC, Darras BT, editors. Neuromuscular Disorders of Infancy, Childhood and Adolescence – A Clinician's Approach. UK: Butterworth-Heinemann; 2003. p. 167-81.
Ochi H, Murai H, Osoegawa 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.
Hirayama K, Toyokura Y, Tsubaki T. Juvenile muscular atrophy of unilateral upper extremity: A new clinical entity. Psychiatr Neurol Jpn 1959;61:2190-7.
Aundhakar,S. C. , Mahajan,S. K. and Chhapra,D. A. (2017). Hirayama's Disease: A Rare Clinical Variant of Amyotrophic Lateral Sclerosis. Advanced Biomedical Research, 2017(july), 1-3.
MLA
Aundhakar,S. C. , , Mahajan,S. K. , and Chhapra,D. A. . "Hirayama's Disease: A Rare Clinical Variant of Amyotrophic Lateral Sclerosis", Advanced Biomedical Research, 2017, july, 2017, 1-3.
HARVARD
Aundhakar S. C., Mahajan S. K., Chhapra D. A. (2017). 'Hirayama's Disease: A Rare Clinical Variant of Amyotrophic Lateral Sclerosis', Advanced Biomedical Research, 2017(july), pp. 1-3.
CHICAGO
S. C. Aundhakar, S. K. Mahajan and D. A. Chhapra, "Hirayama's Disease: A Rare Clinical Variant of Amyotrophic Lateral Sclerosis," Advanced Biomedical Research, 2017 july (2017): 1-3,
VANCOUVER
Aundhakar S. C., Mahajan S. K., Chhapra D. A. Hirayama's Disease: A Rare Clinical Variant of Amyotrophic Lateral Sclerosis. Advanced Biomedical Research, 2017; 2017(july): 1-3.