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Case Report
What a pain in the neck: Crowned dens syndrome as an uncommon cause of neck pain
1 Internal Medicine Resident, VCU Health System, Richmond, Virginia, USA
2 Perlin Professor of Medicine and Health Administration, Department of Internal Medicine, Virginia Commonwealth University, Richmond, Virginia, USA
Address correspondence to:
Alexander M Pandelidis
Department of Internal Medicine, VCU Health System, Richmond, Virginia 23298-0509,
USA
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Article ID: 100064Z06AP2019
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Pandelidis AM, Dow AW. What a pain in the neck: Crowned dens syndrome as an uncommon cause of neck pain. Case Rep Int 2019;8:100064Z06AP2019.ABSTRACT
Introduction: Crowned Dens Syndrome is an uncommon manifestation of calcium pyrophosphate deposition disease caused by crystal deposition and associated inflammation in the C1 and C2 area of the spine. Crowded Dens Syndrome typically causes posterior neck pain and limitation in range of motion. This report describes a case of this unusual and challenging diagnosis and its importance in the differential diagnosis of neck pain.
Case Report: An 80-year-old woman presented as a transfer from another hospital for evaluation for possible epidural abscess. She noted several weeks of progressive posterior cervical neck pain. The pain was worse with passive motion, especially flexion, and she had decreased range of motion in all directions. She had elevated inflammatory markers, and neck computed tomography (CT) scan was non-diagnostic. Broad-spectrum antibiotics for treatment of a possible abscess or meningitis were initiated without improvement. After arrival at our hospital, a dual-energy CT scan concentrated at the C1–C2 area was performed. This imaging revealed multiple calcific deposits surrounding the odontoid with associated soft tissue thickening consistent with Crowned Dens Syndrome due to calcium pyrophosphate deposition disease. She improved with steroid treatment.
Conclusion: Crowned Dens Syndrome presents similarly to infectious, malignant, and other inflammatory causes of neck pain. Because specific imaging is often necessary to make the diagnosis, clinical awareness and suspicion are essential for identifying this condition and prescribing the correct therapy.
Keywords: Calcium pyrophosphate deposition disease, Crowned dens, Neck pain
SUPPORTING INFORMATION
Author Contributions
Alexander M Pandelidis - Substantial contributions to conception and design, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Alan W Dow - Substantial contributions to conception and design, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Guaranter of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient for publication of this article.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors declare no conflict of interest.
Copyright© 2019 Alexander M Pandelidis et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.
