Hirayama disease successfully treated by posterior fixation: a case report

Hirayama disease is a rare form of motor neuron amyotrophy that usually presents with weakness and atrophy of the distal upper extremities in young males. It is believed that it is caused by spinal cord compression during neck flexion because of the widening of the posterior extradural space. This case has been brought to attention due to its extraordinary rarity, serving as an educational tool for medical professionals and to advocate for surgical intervention when deemed necessary.

The authors present a case of a young male in his 20s who was diagnosed with Hirayama disease, had weakness and atrophy in both of his upper limbs, and has been successfully treated by posterior fixation at C4, C5, and C6 with lateral mass screws.

The majority of cases stabilize after 2-3 years of progression; therefore, cervical collars are generally sufficient for therapy. However, in certain serious cases with progression even after that time, surgical intervention is an option. Because this is such an uncommon incidence, surgical therapy has not been explored and is controversial.

The use of posterior fixation at C4, C5, and C6 with lateral mass screws as a therapy for Hirayama disease may be regarded as a successful approach.