OBJECTIVE To describe the clinical and radiological 2-yr followup of 22 patients with anterior atlantoaxial subluxation (AAS) from a prospective cohort of patients with ankylosing spondylitis. METHODS The 2-yr assessment included a structured questionnaire for rheumatologic and neurologic complaints and lateral cervical radiographs in maximal flexion view. Initial and 2-yr radiographs were assessed blind to patient data. The course of anterior AAS was classified as unchanged (< 1 mm), progression (> or = 1 mm) or regression (> or = 1 mm) at 2 yrs compared with baseline. Vertical AAS was classified using the Sakaguchi-Kauppi method. Magnification factor was corrected using the ratio of C3 width. RESULTS Anterior AAS was detected in 22 patients at baseline examination. Two patients also had vertical AAS; 86% were male. Mean age was 33 +/- 9 yrs and mean disease duration was 12 +/- 7 yrs. At followup, one patient had died of acquired immunodeficiency syndrome, 3 could not be reached, and 2 had undergone surgical fusion due to severe myelopathy and now showed complete neurological recovery. Of the remaining 16 patients, 7 (32%) showed progression and 9 (41%) showed no change in the C1-odontoid distance. Vertical AAS developed in one patient. After the 2-yr assessment, 3 additional patients had surgical fusion because of notable progression of AAS, despite absence of neurological signs. CONCLUSIONS Anterior AAS progressed in a number of these patients in the 2 yrs following its detection, and with or without neurological signs, surgical management was thought appropriate in a considerable number of them.