Hirayama disease: Study of clinical, electrophysiological & radiological characteristics at tertiary care centre from north-west India.

Introduction Dynamic cervical cord compression as the pathogenetic mechanism of Hirayama disease has been recently evaluated on dynamic magnetic resonance imaging of cervical spine. Objectives To study clinical, radiological and electrophysiological profile of patients with Hirayama disease. Materials and Methods This was a prospective study of 17 cases of Hirayama disease carried out at SMS hospital, Jaipur, from June 2016 to October 2017. Results In our study, predominantly male (94.1%) patients were affected and average age of onset was 23.1 years. Unilateral involvement was seen in 71% cases (n = 12). Brisk deep tendon reflexes and dysesthesia, not characterstics of Hirayama disease, were found in 3 patients (18%) and 2 patients (12%) respectively. Long neck was found in our study in 7 patients (41%). On dynamic cervical spine MRI, anterior shifting of posterior cervical dura on flexion, asymmetric cord flattening and loss of cervical lordosis were found in 82% patients. Nerve conduction study was abnormal in 14 patients (82%) and electromyographic evidence of neurogenic affliction was present in all patients in involved segments (C5-T1). Conclusion Hirayama disease, a benign variant of motor neuron disorder can be diagnosed with increasing sensitivity with use of dynamic cervical MRI spine study. [ABSTRACT FROM AUTHOR]