Bilateral familial Hirayama disease in a father and daughter
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This article reports a case of bilateral familial Hirayama disease where a father and daughter are the affected members of the family with the similar distribution of their weakness and wasting.

An 18-year-old girl had progressive weakness and wasting of left hand and forearm for 2 years which slowly progressed to involve right hand also. She also had action tremor of both hands (left >right) for a year. On examination, she had minipolymyoclonus and asymmetrical wasting of bilateral forearm and hand with relative sparing of brachioradialis (oblique atrophy). On muscle power testing, there was the weakness of long finger flexors and small muscles of the hand. There was a history of similar illness in the father that started in the left hand at the age of 17 years and a year later involved both hands. His symptoms progressed for 4-5 years and then became stagnant without any specific treatment. Father had a more severe wasting of both forearms and hands with sparing of brachioradialis, diminished deep tendon reflexes in the upper extremities, and minipolymyoclonus in both hands. Sensory examination was normal in father and daughter. Their MRI of spine and electromyography were suggestive of Hirayama disease. The amplitude of the compound motor action potential was reduced in father indicating more severe involvement.

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