A case of monomelic amyotrophy of the upper limb: MRI findings and the implication on its pathogenesis

Monomelic amyotrophy of the upper limb or Hirayama disease is mostly considered as an anterior horn disorder resulting from local ischemia, triggered by arterial compression from an anterior shifting of the posterior cervical dura upon neck flexion. However, such a dural shifting is not universally seen. We report on a Caucasian male patient who developed a slowly progressive unilateral distal hand weakness in his teens. His clinical and electromyographic findings were consistent with Hirayama disease. Local anterior cervical cord atrophy was observed without dural shifting on the dynamic magnetic resonance imaging. Axial magnetic resonance imaging demonstrated signal changes of "snake-eye" appearance in the cervical anterior horn region, similar to ischemic myelopathies caused by various etiologies. This case illustrated that even without dural shifting, a mechanism of anterior spinal cord ischemia could still be responsible for the pathogenesis of Hirayama disease.