Vol. 3, Issue 3 (2018)
Clinicoradiological profile of Hirayama disease: An experience from a tertiary care centre in Kerala
Author(s): Muhammad PK, Vishal VP, Shaji CV, Kabeer KA, Nikhil G, Meenakumari S
Abstract: Background: Hirayama disease (HD) is a benign disease characterized by unilateral or asymmetric distal upper limb weakness and wasting. Objective: To study the clinical and radiological profile of patients with HD. Materials and Methods: Clinicoradiological evaluation were done in a total of 8 patients presented with insidious‑onset hand wasting from March 2015 to June 2017. Results: All were males, mean age of onset of 19.5 years. 7 out of 8 (87.5%) had asymmetric wasting and weakness. All (100%) had oblique amyotrophy, two (25%) had cold paresis, four (50%) had minipolymyoclonus, and 3 (37.5%) had history of trauma to cervical region. Flexion MRI contrast study showed enchancing epidural crescent with anterior displacement of posterior cervical dura in all patients. Conclusion: Though Dynamic contrast MRI is characteristic, routine studies have a high predictive value for diagnosis. Prompt diagnosis is important to institute early collar therapy.