The Clinical Spectrum of Crowned Dens Syndrome: An Analysis of Seven Cases

The crowned dens syndrome is an unusual manifestation of calcium pyrophosphate dihydrate crystal deposition disease and represents a rare cause of neck pain. We report seven cases of crowned dens syndrome, assessing clinical and radiological features, associated conditions, therapy, and outcome. The acute presentation of crowned dens syndrome raised suspicion for infectious meningitis and cranial giant cell arteritis in two cases. Cervical cord compression was the initial presentation of periodontoid calcium pyrophosphate deposition disease in two other cases. A prior history or concurrent, self-limited, peripheral arthritis was clinically or radiologically apparent. Computed tomography scans performed better than magnetic resonance imaging in assessing calcifications of the dens area. We found a unique association with diffuse idiopathic squeletal hyperostosis, Eagle’s syndrome, and Paget’s disease of bone. Prompt initiation of therapy including isolated or combinations of non-steroidal anti-inflammatory drugs, corticosteroids, and colchicine yielded a dramatic response.