Subacute onset Hirayama disease with extensive myelopathy on MRI in flexed posture

Excerpt

Our case is an exceptional illustration of the proposed flexion-induced pathophysiology of Hirayama disease. Evidence, privileging a mechanical pathophysiology, is growing and diagnostic inclusion criteria are involving MRI-confirmed evidence of the mechanical pathophysiology. The forward displacement of the posterior wall during neck flexion is considered to be the hallmark and primary pathophysiologic mechanism of this disease [1]. In our case, the subacute onset and short disease course with contrasting extensiveness of the myelopathy could be explained by a more important contribution of secondary ischemic myelopathy with subsequent extensive T2-hyperintensity and clinical pyramidal signs. We support that the technical diagnostic workup of Hirayama disease primarily includes dynamic MRI imaging of the cervical spine next to MRI imaging in neutral position, as suggested by Huang and Chen [2]. …