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A Royal Pain in the Neck: Crowned Dens Syndrome Mi ...
A Royal Pain in the Neck: Crowned Dens Syndrome Mimicking Meningitis
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A 58-year-old man with treated HIV, hepatitis C status post liver transplant on cyclosporine, prior Burkitt lymphoma, CKD, atrial fibrillation, and gout presented with 48 hours of fever, headache, and nuchal rigidity after recent dry needling of the neck and back. He also had a swollen, painful right elbow. Because his presentation strongly suggested meningitis, he was admitted and started on broad-spectrum antimicrobials and acyclovir.<br /><br />Initial workup, including lumbar puncture, was negative. Cervical CT and MRI showed calcium deposition around the dens of C2 and pannus formation at C1/C2, findings consistent with crowned dens syndrome (CDS), a rare manifestation of calcium pyrophosphate deposition disease (CPPD). Arthrocentesis of the right elbow revealed intracellular calcium pyrophosphate crystals, confirming pseudogout and supporting the diagnosis.<br /><br />CDS is an underrecognized meningitis mimic that can present with acute neck pain, fever, nuchal rigidity, and elevated inflammatory markers. It is often misdiagnosed, leading to diagnostic delay. In this case, the combination of a high index of suspicion, imaging, and early arthrocentesis allowed diagnosis by hospital day 2.<br /><br />Treatment with prednisone led to rapid improvement and near-complete symptom resolution. Antibiotics were discontinued once infection was excluded. He could not receive colchicine because of interaction with cyclosporine, and NSAIDs were avoided due to CKD and other comorbidities. He was discharged on a short prednisone taper and low-dose prednisone for CPPD prophylaxis.<br /><br />This case highlights the importance of considering crowned dens syndrome when meningitis evaluation is negative but severe neck pain and rigidity persist, especially in patients with risk factors for CPPD. Early spinal imaging can shorten hospitalization and prevent unnecessary treatment.
Asset Subtitle
Lila M. Lehtola
Meta Tag
Author List
Henry R. Kramer, Lila M. Lehtola, Matthew Wofford
Category
Clinical Vignettes
Concept
Crowned Dens Syndrome
Concept
Neck Pain
Concept
Nuchal Rigidity
Concept
Pseudogout
Concept
Fever
Distinguished
Non-Finalist
Presenter Organization
University of Colorado Hospital--Anschutz
Presenting Author
Lila M. Lehtola
Track
Adult
Keywords
crowned dens syndrome
calcium pyrophosphate deposition disease
pseudogout
meningitis mimic
neck pain
nuchal rigidity
cervical CT
prednisone
arthrocentesis
immunosuppressed patient
Crowned Dens Syndrome
Neck Pain
Nuchal Rigidity
Pseudogout
Fever
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