Crowned dens syndrome — SCE Rheumatology MCQ

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EasyCrystal arthropathiesCrowned dens syndromeSCE Rheumatology

An 82-year-old woman presents with sudden severe upper neck pain, feverish malaise and marked restriction of neck rotation. There is no focal neurological deficit. CT of C1–C2 shows curvilinear calcification in the peri-odontoid ligaments surrounding the dens. What is the most likely diagnosis?

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Correct answer: D — CPPD crowned dens syndrome

The diagnosis is **CPPD crowned dens syndrome**. It results from calcium pyrophosphate crystal deposition in the transverse and other peri-odontoid ligaments. The characteristic presentation is acute severe upper cervical pain and stiffness, often with fever and raised inflammatory markers, in an older adult. Markedly painful restriction of neck rotation and crown-like peri-odontoid calcification on CT are particularly discriminating features. Meningitis can produce fever and neck stiffness but does not explain the characteristic ligamentous calcification. A cervical disc prolapse more often causes radicular pain or neurological signs and is not associated with fever. Giant cell arteritis requires compatible cranial or ischaemic features, while ankylosing spondylitis is a chronic inflammatory condition that usually begins much earlier in life.

Reference: Hamdan A, et al. Clinical features and diagnostic challenges in crowned dens syndrome: a systematic review and meta-analysis. Rheumatology International. 2025. https://pubmed.ncbi.nlm.nih.gov/40278908/