CASE HISTORY – February 2023

Hirayama’s disease

Authors: Triin Helin Unt

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Hirayama’s disease, or Hirayama syndrome,is a rare nonfamiliar monomelic amyotrophy that classically manifests itself as muscle
atrophy and weakness of unilateral or bilateral forearms and hands in absence of sensory alterations. The complaints may worsen for 3-5 years until a plateau state is achieved. Hirayama’s disease was first described by Hirayama and co-authors in 1959 in a Japanese patient presenting with unilateral distal atrophy of the upper limb. The condition is thought to be caused by a tight dural sac in the cervical canal, leading to chronic ischaemic changes in the anterior horn nerve cells. Although Hirayama’s disease is generally considered to be a self-limiting disease, it may cause significant disability in affected patients. Early diagnosis and intervention can limit the progression of the disease and thereby reduce the severity of disability.