Crowned dens syndrome: a manifestation of hydroxy-apatite rheumatism

Abstract

Crowned dens syndrome is defined as an association of acute cervical pain and calcifications in the peri-odontoid space. The authors report one case of this rare localization of hydroxy-apatite rheumatism and review 12 similar cases in the literature. This disease affects only adult females. Patients present with inflammatory signs, can be treated with non-steroid anti-inflammatory drugs and recover without sequela. Calcium pyrophosphate dihydrate deposition can also lead to this syndrome. Other perioodontoid calcifications and ossifications, usually asymptomatic, appear only as a radiologically crowned dens.

Pseudogout Showing Meningoencephalitic Symptoms: Crowned Dens Syndrome

“…The deposition of CPPD crystals was confirmed by fluid aspiration of the right knee joint. In general, the clinical course of crowned dens syndrome by CPPD deposition is less uniform than HA cases (5). The number of reported cases of male patients with crowned dens syndrome caused by CPPD is much less than that for females (3,5).…”

“…The crowned dens syndrome is defined as a combination of acute feverish cervical pain and calcification surrounding the odontoid process (4,5). These clinical manifestations could be caused by HA and/or CPPD depositions (1).…”

“…Severe neck pains and occipitalgia sometimes occur in patients with hydoxyapatite (HA) and calcium pyrophosphate dehydrate (CPPD) crystal deposition diseases (1,2). The crowned dens syndrome has been commonly termed as acute neck pain attributed to HA and/or CPPD depositions surrounding the odontoid process (3)(4)(5). We encountered a patient with severe occipitalgia, cervical rigidity and a high fever, all of which suggested meningitis.…”

The crowned dens syndrome as a cause of neck pain: Report of two new cases and review of the literature

“…CDS was described by Bouvet et al in 1985 as neck pain caused by deposition of hydroxyapatite or CPPD crystals in the cervical spine and appearing radiographically as radioopaque densities surrounding the top and sides of the odontoid process in a crown-or halo-like distribution (11). In addition to the 4 cases described by Bouvet and the 2 cases presented in this series, we identified 11 additional cases in the English literature via Medline and PubMed database searches and citations from relevant articles (11)(12)(13)(14)(15). All patients presented with neck pain though duration of symptoms ranged from acute to several years.…”

Cervical spine and crystal-associated diseases: imaging findings

“…However, the histological study of the crystals is the reference to assess the definite diagnosis. The modification or resorption of the calcifications is common and may be complete in 2 weeks [14]. The open mouth view can show the heterogeneous deposits around the dens, but osseous superimpositions are frequent.…”