Grisel's syndrome. Cervical spine clinical, pathologic, and neurologic manifestations

Grisel's syndrome involves the subluxation of the atlantoaxial joint from inflammatory ligamentous laxity following an infectious process. Even though it was first described in 1830, it is a rare disease usually affecting children, but infrequent adult cases do occur. Patients generally seek treatment for progressive unrelenting throat and neck pain followed by torticollis and subluxation. Neurologic complications occur in approximately 15% of cases and can range from radiculopathy to myelopathy and even death. Principles of management include bacteriologic cure and correction of bony deformity and neurologic protection. The authors present two adult patients with Grisel's syndrome. The first illustrates the permanent spinal deformity that can occur if the disease remains unrecognized for a prolonged period of time. The second case demonstrates a delayed neurologic complication in an adult who had Grisel's syndrome in childhood. It is hoped that these two examples, together with a detailed discussion of the literature, will inform physicians of an unusual but important condition to be considered in the differential diagnosis of any patient complaining of neck pain.