Severe occipital pain caused by periodontoid calcifications: Crowned dens syndrome

Abstract

Keywords

Neck stiffness meningitis cervical arthropathy

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).

Footnotes

Funding

This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Conflict of interest

None declared.

References

Aouba

Vuillemin-Bodaghi

Mutschler

De Bandt

. Crowned dens syndrome misdiagnosed as polymyalgia rheumatica, giant cell arteritis, meningitis or spondylitis: An analysis of eight cases. Rheumatology (Oxford) 2004; 43: 1508–1512.

Bouvet

le Parc

Michalski

. Acute neck pain due to calcifications surrounding the odontoid process: The crowned dens syndrome. Arthritis Rheum 1985; 28: 1417–1420.

Siau

Lee

Laversuch

. Acute pseudogout of the neck–the crowned dens syndrome: 2 case reports and review of the literature. Rheumatol Int 2011; 31: 85–88.