Crowned Dens Syndrome: A Case Report and Review of the Literature

Abstract

The crowned dens syndrome (CDS), also known as periodontoid calcium pyrophosphate dehydrate crystal deposition disease, is typified clinically by severe cervical pain, neck stiffness and atlantoaxial synovial calcification which could be misdiagnosed as meningitis, epidural abscess, polymyalgia rheumatica, giant cell arthritis, rheumatoid arthritis, cervical spondylitis or metastatic spinal tumor. Crystalline deposition on cervical vertebrae is less well known disease entity and only a limited number of cases have been reported to date. Authors report a case of CDS and describe the clinical feature.

Crowned Dens Syndrome as an Initial Manifestation of Crystalline Deposition Disease

“…The appropriate treatment for CDS remains debatable; however, NSAIDs are conventionally used for CDS (3,5,8). In most of the previously reported cases, oral NSAIDs therapy alone was sufficient for the improvement of symptoms within several days.…”

Usefulness of cervical computed tomography and magnetic resonance imaging for rapid diagnosis of crowned dens syndrome: A case report and review of the literature

“…CDS is a rare disease entity which radiologically shows calcification of the cruciform ligament around the odontoid process and clinically presents with acute cervico-occipital pain, fever, and neck stiffness accompanied by a biological inflammatory reaction [1], [2], [3], [4], [5]. The diagnosis of CDS is based on the combination of clinical, biological, radiological, and therapeutic findings: acute periodic attacks of feverish cervico-occipital pain and neck stiffness with biological inflammatory syndrome, radiological identification of a crown-like calcification surrounding the odontoid process due to microcrystalline deposits, and a drastic improvement in symptoms under treatment with NSAIDs or colchicine [1], [5], [6], [7].…”

“…Therefore, CDS should be considered in the differential diagnosis of fever, headache, and neck pain of unknown origin [3], [5], [6]. ; Cervical CT scanning focusing on C1 and C2 is the gold standard of CDS diagnosis [1], [2], [3], [4], [5], [6], [7], [8]. The typical tiny, half-ringed, crown-like form of calcification behind the dens is described as the most important and definitive feature of CDS.…”

“…Crowned dens syndrome (CDS) is a radioclinical entity defined by the association of microcrystalline deposition of calcium pyrophosphate dehydrate (CPPD) in the cruciform ligament around the odontoid process and is demonstrated radiologically as a radiopaque density in a crown or halo-like distribution that appears at the superior and lateral portions of the odontoid process [1], [2], [3], [4]. The deposits of CPPD can remain asymptomatic or can be responsible for chronic cervical illness or spinal cord compression and lead to periodic acute cervico-occipital pain with fever, neck stiffness, and biological inflammatory syndrome [1], [5].…”

Crowned dens syndrome

“…Tumors and acute calcific retropharyngeal tendinitis may also exhibit dens-associated calcium deposition [6]. Crowned dens syndrome can mimic meningitis, epidural abscess, rheumatoid arthritis, metastatic spinal tumor, and other conditions [7], leading to misdiagnosis, unnecessary invasive procedures, inappropriate treatment, and prolonged hospitalization [8]. Typically, a nonsteriodal antiinflammatory drug and/or low-dose steroid treatment achieves quick resolution [5].…”