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Hirayama Disease
What is Hirayama Disease?
Hirayama disease, also known as monomelic amyotrophy, Sobue disease or Juvenile non-progressive amyotrophy is a rare and incurable disease that was first described in 1959 by Dr. Keizo Hirayama, a neurologist, attached to the Chiba University School of Medicine in Japan. It mainly affects young males aged between 15-25 years, generally after they experience their adolescent growth spurt. Technically, it is termed as a lower motor neuron disorder.
Classical findings include muscle wasting (atrophy
) and weakness of the forearms and hands, either on one or both sides. There is, however, no change in sensation and the patient doesn’t experience any pain. There may be involuntary muscle twitching (fasciculations), but these are rare. The disease usually progresses for 2-5 years before stabilizing or plateauing. After plateauing, improvement in the symptoms does not occur. However, it does not worsen either. ....

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