A Study of Diffusion Tensor Imaging in Hirayama Disease

Background: Hirayama disease (HD) is a motor neuron disease and occasionally is associated with lower limb hyper-reflexia. Corticospinal tract dysfunction can be evaluated by diffusion tensor imaging (DTI), but there is paucity of study in HD. Objective: We report corticospinal tract functions using DTI in the patients with HD and correlate with clinical findings. Materials and Methods: The patients with HD diagnosed on the basis of clinical and electromyography findings were included. Their age, duration of illness, side of initial involvement, and progression were noted. Presence of lower limb hyper-reflexia, and cervical spine magnetic resonance imaging (MRI) findings were noted. Cranial MRI was done and DTI findings at internal capsule, cerebral peduncle, pons, and pyramid were noted. Results: In total, 10 patients with HD and 5 matched controls were evaluated. The apparent diffusion coefficient (7.03 +/- 0.27 vs 6.83 +/- 0.36), fractional anisotropy (0.79 +/- 0.04 vs 0.82 +/- 0.05), axial diffusivity (5.08 +/- 0.08 vs 5.04 +/- 0.07), and radial diffusivity (3.79 +/- 0.05 vs 3.76 +/- 0.05) between HD patients and controls were not different in internal capsule. These values were also not significantly different in cerebral peduncle, pons, and pyramid. These values were also not significantly different between the severe and less severely affected sides. The fractional anisotropy did not correlate with lower limb hyper-reflexia (P = 1.00) and spinal cord atrophy (P = 0.60). Conclusion: DTI study in HD patients did not reveal corticospinal tract involvement in brain.