Abstract
C1 stenosis is often an easily missed cause for cervical myelopathy. The vast majority of cervical myelopathy occurs in the subaxial cervical spine. The cervical canal is generally largest at C1/2, explaining the relatively rare incidence of neurological deficits in patients with odontoid fractures. However, some subjects have anatomical anomalies of the atlas, which may cause stenosis and result in clinical symptoms similar to subaxial cord compression. Isolated pure atlas hypoplasia leading to stenosis is quite rare and may be associated with other anomalies, such as atlas clefts or transverse ligament calcification. It may also be more commonly associated with syndromic conditions such as Down or Turner syndrome. Although the diagnosis can be easily made with a cervical magnetic resonance imaging, the C3/2 spinolaminar test using a lateral cervical plain radiograph is a useful and sensitive tool for screening. Surgical treatment with a C1 laminectomy is generally necessary and any atlantoaxial or occipito-atlanto instability must be treated with spinal stabilization and fusion.