BACKGROUND: Patients with crowned dens syndrome (CDS), which is pseudogout of 
the atlantoaxial junction induced by "crown-like" calcifications around the 
dens, present with symptoms of severe neck pain, rigidity, and high fever. CDS 
patients are often misdiagnosed as having meningitis or polymyalgia rheumatica, 
leading to potentially unnecessary invasive procedures for diagnosis and 
treatment.
CASE REPORT: We report 3 patients with CDS who had characteristic findings on 
computed tomography (CT), all of whom quickly recovered with nonsteroidal 
antiinflammatory drug (NSAID) administration. In addition, we reviewed 72 
published cases, including our patients. CDS typically occurs in elderly people 
(mean age 71.4 years). Common symptoms include neck pain (100%), neck rigidity 
(98%), and fever (80.4%), and most show elevated inflammatory markers (88.3%) on 
serum laboratory tests. Neck pain on rotation is a characteristic and helpful 
symptom in the diagnosis. The most useful modality is CT (97.1%), showing linear 
calcium deposits around the dens, mostly in the transverse ligament of atlas 
(TLA). CT number is especially helpful to distinguish a normal TLA (35-110 HU) 
from a calcified one (202-258 HU) in our cases. The most effective treatment is 
NSAID administration (85%), which usually leads to marked resolution of symptoms 
within days or weeks. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Due 
to acute and severe symptoms, CDS patients often present to an emergency 
department. To avoid unnecessary invasive procedures for diagnosis and 
treatment, CDS should be considered in the differential diagnosis of febrile 
neck pain.