In this study, we used a multiparametric MR imaging approach to assess a patient with Hirayama disease (HD). We found that cervical cord damage extends beyond cord T2-visible lesions. We also showed an altered pattern of cortical activations during movements of clinically unaffected limbs. Whereas this study suggests a more widespread cord involvement in HD than seen on routine MR imaging, its cause remains unclear (vascular damage versus a primary lower motor neuron disease).