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Updated: Feb 24, 2026

Utility of Dissociated Intrinsic Hand Muscle Atrophy in the Diagnosis of Amyotrophic Lateral Sclerosis
Published on: March 4, 2014
Hirayama's Disease: A Rare Clinical Variant of Amyotrophic Lateral Sclerosis
Swati C Aundhakar1, Sanket K Mahajan1, Daanish A Chhapra1
1Department 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.
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