Severe occipital pain caused by periodontoid calcifications: Crowned dens syndrome

A 76-year-old woman presented to the emergency department with severe occipital pain and fever. Neurologic examination showed neck tenderness and stiffness. Laboratory evaluation showed an ESR of 50 mm/hour (normal: <30 mm/hour) and a CRP level of 5.2 mg/dl (normal: <0.3 mg/dl). Diagnostic workup showed no evidence of meningitis or rheumatoid arthritis. Cervical computed tomography (CT) scan revealed periodontoid calcifications (Figure 1) suggesting a clinical diagnosis of cervical localization of crystal-related arthropathy; crowned dens syndrome (CDS) (1,2). Treatment with 2400 mg/day acetaminophen resulted in complete resolution of pain within a week. She was discharged on hospital day 11 and returned to her physician for follow-up.

Figure 1.

Cervical CT scan revealing periodontoid calcifications in axial (a) and sagittal views (b) (white arrowheads).

CDS is emerging as a better delineated condition and should be included in the differential diagnosis of cervico-occipital pains in association with fever, neck stiffness and raised inflammatory markers (3).