Diffusion Tensor Imaging Characteristics in Hirayama Disease: Case Report and Review of the Literature.

Abstract

Hirayama disease (HD) is a rare focal amyotrophy, commonly reported in adolescent Asian males and characterized by unilateral or asymmetric distal upper extremity weakness. This disease exhibits an indolent course of progression over the initial 3-4 years, followed by a stage of stabilization. The diagnosis is confirmed by demonstration of enhanced posterior epidural space and dynamic spinal cord compression on cervical flexion magnetic resonance imaging. Diffusion tensor imaging (DTI) is a functional imaging modality, which can potentially be utilized in the diagnosis of HD. We hereby report 2 young male patients who had presented to us with bilateral asymmetric upper extremity weakness. In both patients, magnetic resonance imaging of cervical spine revealed spinal cord atrophy or myelomalacia, anterior displacement of posterior thecal sac, and T2 hyperintense posterior epidural space, all of which were more evident in flexed position. On DTI, we could observe reduced fractional anisotropy in cervical flexion, as compared with neutral. DTI tractography and reduced fractional anisotropy in cervical flexion can serve as potential indicators for the diagnosis of HD. Large-scale studies in the future are necessary to identify the potential role of this modality in surgical decision-making and in the assessment of prognosis in patients with HD.