Cervical CT-Dependent Diagnosis of Crowned Dens Syndrome in Calcium Pyrophosphate Dihydrate Crystal Deposition Disease

Fever of Unknown Origin with Fluorodeoxyglucose-crowned Dens Syndrome on Positron Emission Tomography: Case Reports

“…8 The prevalence of "concomitant" periodontoid calcifications with or without neck symptoms in CPPD patients has been reported as 51% to 63%. 4,6,8 These prevalence data in non-CPPD and CPPD patients highlight the importance of other criteria such as pyrexia or inflammatory markers in making a diagnosis of CDS, especially in the elderly people. 7 The use of FDG to image infection and inflammation is widely accepted.…”

“…Approximately 320 cases were found in the English literature. It remains a clinicoradiological entity but with a widening spectrum, characterised by locoregional features (e.g., neck pain, neck rigidity, headache, shoulder pain), inflammatory response (pyrexia, leucocytosis, elevated erythrocyte sedimentation rate, C-reactive protein level), and calcium crystal deposition at and around the atlantoaxial articulations evident as periodontoid calcifications on CT. 1,2,[4][5][6] Nonetheless there is no consensus on diagnostic or inclusion criteria, hence a wide variation of reported presentations, e.g., local symptom onset varies as acute, subacute, chronic, periodic or uncertain; pyrexia is present or absent; inflammatory markers are normal or elevated; and rare features such as meningeal signs or cervical myelopathy. FUO is also rarely reported, mimicked by cases of prolonged evolution with relapses.…”

“…It may have led to better recognition of CDS, even when asymptomatic, in patients with CPPD disease. 4,6 Nonetheless such CT findings may also be common in the elderly people with no CPPD disease history. The prevalence of "incidental" periodontoid calcifications on CT has been reported in the United States to be 34% in those aged ≥60 years, and 49% for those aged ≥80 years.…”

Cervical myelopathy due to subaxial calcium pyrophosphate dihydrate (CPPD) deposition with simultaneous asymptomatic crowned dens syndrome: two case reports

“…CT scans, the most diagnostic examination, demonstrate oval-shaped calcified lesions anterior to the laminae with clear margins [ 22 , 23 ]. The lesions are observed centrally, not laterally, beneath the laminae.…”

Crowned dens syndrome

“…CDS is considered an underdiagnosed, yet typical presentation of calcium pyrophosphate deposition disease (CPDD) as the second most common crystal-induced arthritis. 1 Therapeutic strategies lean on gout management, including nonsteroidal anti-inflammatory drugs (NSAIDs), colchicine, or low-dose steroids in chronic kidney disease.…”