• J Emerg Med · Jul 2015

    Review Case Reports

    Crowned Dens Syndrome: Report of Three Cases and a Review of the Literature.

    • Akihiko Oka, Koichi Okazaki, Ayumu Takeno, Satoshi Kumanomido, Ryusaku Kusunoki, Shuichi Sato, Shunji Ishihara, Yoshikazu Kinoshita, and Masayoshi Nishina.
    • Department of Emergency and Critical Care Medicine, Faculty of Medicine, Shimane University, Enya-cho, Izumo-City, Shimane Prefecture, Japan.
    • J Emerg Med. 2015 Jul 1;49(1):e9-e13.

    BackgroundPatients 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 ReportWe 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.Copyright © 2015 Elsevier Inc. All rights reserved.

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