A Rare Case of Hirayama Disease in a 28-Year-Old Indian Woman

Abstract

A 28-year-old woman presented with slowly progressive weakness of the right upper limb since 14 years of age. This was purely motor, distally located and progressive in nature. It did not involve any other limbs. She also had a history of hypothyroidism and gestational diabetes. On examination, hypothenar muscle wasting and complete claw hand was noted (Figure 1) Power was reduced in muscles of the hand, while reflexes were bilaterally absent on both upper limbs. Distal asymmetric motor neuropathy was suspected, and nerve conduction studies confirmed the same. Plain MR imaging of the whole spine with dynamic flexion and extension of cervical spine was done which showed features consistent with Hirayama disease (Figure 2).