To explore the pathogenesis of Hirayama disease from juvenile cervical curvature and growth rate.

Totally 60 patients diagnosed with Hirayama disease (HD) from 2009 to 2011 in our hospital were included in the present study. Patient's height and growth rate 1-2 years prior to the onset of disease were recalled by patients and family members. Lateral cervical X-ray was examined, and cervical curvature was measured by Borden's method.

All the patients were adolescents with onset age at 12-25 (17.0±2.4) years old and peak age of onset at 15-18 [45 cases (75.0%)]. Fifty-seven cases were male and 3 cases were female. Cervical MRI examination of the 60 cases showed that the spinal cord atrophy involving C4-C8 vertebral level. The C line values for cervical curvature by Borden's method of the patients was 2.6 (1.2, 4.2) mm. Among 60 patients, 57 of them were with abnormal cervical curvature. The average height growth rate 1 year prior to the onset was (7.1±1.8) cm.

The clinical manifestations that featured in overgrowth in the first two years and abnormal cervical vertebra curvature are possible related with pathogenesis of HD. HD is possibly a cervical spinal cord compression disease, which is associated with cervical spinal dysplasia during juvenile growth.

Hirayama disease; Pathogenesis