Calcium Pyrophosphate Deposition Disease in the Craniovertebral Junction: An Unusual Cause of Neck Pain

Abstract

Crowned Dens Syndrome (CDS) is a rare manifestation of Calcium Pyrophosphate Deposition (CPPD) disease characterised by calcification around the odontoid process in the atlantoaxial joint. It manifests as intense neck pain with signs of systemic inflammation, including elevated acute phase reactants. Thus, it can simulate other causes of neck pain and diagnosis can be challenging. Moreover, CPPD disease can be asymptomatic and observed as radiological finding. Here in, two cases of spinal CPPD disease with distinct presentations are reported. The first of 83-year-old woman describes CDS that was misdiagnosed as Polymyalgia Rheumatica (PMR) whereas in the second case of 70-year-old female calcification around the odontoid process was incidentally found in a patient with acute cervical spine fracture. These two reported cases highlight the spectrum of manifestations of spinal CPPD disease and support the notion that careful discrimination of the cause of neck pain is essential for improving outcomes.