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SAJEM. 2019; 2(2): 37-39

Hirayama Disease: A Rare Clinical Entity

Salman Mansoor, Kevin Murphy, Mohammad Hijaz Adenan, Eimear Joyce, Orla Hardiman, Michael Hennessey, Siobhan Kelly.

Hirayama disease is a rare neurological entity which can present in any setting emergency departments included. These cases are usually diagnosed after carefully excluding other conditions. Intraspinal lesions (eg, syringomyelia, syringobulbia, or tumor) can present with symptoms that can mimic hirayama disease. We report the case of a 23-year old man who presented with an 18 month history of numbness, tingling and painless curling of the 4th and 5th digits.The symptoms did not progress beyond the 12 month period. The right side and bulbar function remained unaffected throughout. Based on the age of onset, arrest of progression after a period of deterioration, imaging features and neurophysiological findings a diagnosis of Hirayama Disease was made.

Key words: Hirayama Disease; Monomelic Amyotrophy; Motor neuron disease

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