[A case of Hirayama's disease successfully treated by anterior cervical decompression and fusion]

Abstract

The authors report the case of a 16-year-old boy with Hirayama's disease(juvenile muscular atrophy of unilateral upper extremity). The present history began about 6 months previously, when he noticed slowly progressive weakness with atrophy of the left hand and forearm. Neurological examination on admission revealed diffuse distribution of muscular atrophy including the left hypothenar, thenar, forearm, and triceps muscles. However, EMG studies identified neurogenic changes in both upper extremities. There was no long tract sign of objective sensory impairment. Plain spinal radiograms showed abnormal kyphosis of the cervical vertebrae. Cervical MR images in the neutral position demonstrated focal atrophy of the cervical cord at the C 5-6 vertebral levels. When the neck was flexed, the cervical cord was displaced anteriorly and was compressed over the posterior surface of the C 5-6 vertebral bodies. He was diagnosed to have Hirayama's disease(cervical flexion myelopathy). Via an anterior approach, he underwent a C 5 vertebrectomy followed by fixation of C 4-6 vertebral bodies using iliac bone and plate system. He recovered from surgery without any complications and has been well for the past 6 months with remarkable improvement of muscle strength. Application of cervical collar for 3 to 4 years has been generally advocated for the treatment of Hirayama's disease because progression of signs and symptoms is usually expected to cease within several years. However, some patients were reported not to response to conservative treatment for more than 5 years after their onsets. To these patients surgery seems to be beneficial, because it can give rise to permanent stable fixation with much shorter period of external cervical immobilization compared with cervical collar therapy, in which long-term application is frequently unbearable in many patients. In conclusion, the present case emphasizes the importance of surgical treatment in Hirayama's disease not only to improve neurological deficits but regain better quality of life.