[Skip to Content]
[Skip to Content Landing]

A Man With Headache, Fever, and Neck Stiffness

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 53-year-old man presented with 2 weeks of back pain, stiff neck, headache, and fevers. He also reported 3 days of right knee pain and swelling. He had a history of pseudogout and kidney transplant 13 years ago. He was taking sirolimus and prednisone, with excellent graft function. His temperature on presentation was 102°F (38.9°C). He had nuchal rigidity and positive Kernig sign. There were no focal neurologic deficits and no spinal tenderness. He had a right knee effusion without erythema and with full range of motion. Laboratory workup of serum showed a white blood cell (WBC) count of 10 200/μL (neutrophils, 63.8%; lymphocytes, 19.6%; monocytes, 15.1%) and erythrocyte sedimentation rate, 63 mm/h; workup of cerebrospinal fluid showed a WBC count of 0/μL; red blood cell count, 32/μL; total protein level, 109 mg/dL; and glucose level, 59 mg/dL (3.3 mmol/L). Magnetic resonance imaging (MRI) of the spine showed cervical degenerative changes with reactive marrow (Figure 1). He was prescribed intravenous vancomycin and cefepime. Blood and cerebrospinal fluid (CSF) cultures were negative.

Please finish quiz first before checking answer.

You answered correctly!

Read the answer below and download your certificate.

You answered incorrectly.

Read the discussion below and retake the quiz.

Crowned dens syndrome

B. Obtain additional imaging with computed tomography (CT)

The key to the correct diagnosis is the presence on a CT scan of synovial crown- or halo-like calcific densities of the atlantoaxial (C1/C2) joint, surrounding the odontoid process, in a patient with history of peripheral acute calcium pyrophosphate (CPP) crystal arthritis (“pseudogout”) (Figure 2). This patient had crowned dens syndrome (CDS), ie, deposition of CPP or, less frequently, basic calcium phosphate (BCP) crystals in the atlantoaxial joint, causing symptoms and signs of cervical spine inflammation. Continuation of antibiotics was not indicated, since CSF and MRI findings were not consistent with infection. Gabapentin is effective against neuropathic but not acute inflammatory pain. Like other forms of crystal deposition disease, CDS can be treated with corticosteroids or colchicine when there is a relative contraindication to use of nonsteroidal anti-inflammatory drugs (NSAIDs, eg, ibuprofen), such as renal transplant in this patient.

Survey Complete!

Sign in to take quiz and track your certificates

Buy This Activity

JN Learning™ is the home for CME and MOC from the JAMA Network. Search by specialty or US state and earn AMA PRA Category 1 CME Credit™ from articles, audio, Clinical Challenges and more. Learn more about CME/MOC

Article Information

Corresponding Author: Dimitrios Farmakiotis, MD, Rhode Island Hospital, 593 Eddy St, Gerry House 111, Providence, RI 02903 (Dimitrios.Farmakiotis@lifespan.org).

Published Online: March 29, 2019. doi:10.1001/jama.2019.1772

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for providing permission to share his information.

References
1.
Matsumura  M, Hara  S.  Images in clinical medicine: crowned dens syndrome.  N Engl J Med. 2012;367(23):e34. doi:10.1056/NEJMicm1100764PubMedGoogle ScholarCrossref
2.
Le Goff  P, Pennec  Y, Schwarzberg  C.  Acute febrile neck pain simulating a meningeal syndrome, disclosing articular chondrocalcinosis  [in French].  Rev Rhum Mal Osteoartic. 1980;47(7-9):507-509.PubMedGoogle Scholar
3.
Scutellari  PN, Galeotti  R, Leprotti  S,  et al.  The crowned dens syndrome: evaluation with CT imaging.  Radiol Med. 2007;112(2):195-207. doi:10.1007/s11547-007-0135-7PubMedGoogle ScholarCrossref
4.
Sekijima  Y, Yoshida  T, Ikeda  S.  CPPD crystal deposition disease of the cervical spine.  J Neurol Sci. 2010;296(1-2):79-82. doi:10.1016/j.jns.2010.05.028PubMedGoogle ScholarCrossref
5.
Aouba  A, Vuillemin-Bodaghi  V, Mutschler  C, De Bandt  M.  Crowned dens syndrome misdiagnosed as polymyalgia rheumatica, giant cell arteritis, meningitis or spondylitis: an analysis of eight cases.  Rheumatology (Oxford). 2004;43(12):1508-1512. doi:10.1093/rheumatology/keh370PubMedGoogle ScholarCrossref
6.
Ea  HK, Lioté  F.  Diagnosis and clinical manifestations of calcium pyrophosphate and basic calcium phosphate crystal deposition diseases.  Rheum Dis Clin North Am. 2014;40(2):207-229. doi:10.1016/j.rdc.2014.01.011PubMedGoogle ScholarCrossref
7.
Chang  EY, Lim  WY, Wolfson  T,  et al.  Frequency of atlantoaxial calcium pyrophosphate dihydrate deposition at CT.  Radiology. 2013;269(2):519-524. doi:10.1148/radiol.13130125PubMedGoogle ScholarCrossref
8.
Sano  M, Yamashita  S, Aiba  T.  The prevalence of calcification around odontoid process and the incidence of crowned dens syndrome in the neurosurgical ward.  Mod Rheumatol. 2018;28(1):182-187. doi:10.1080/14397595.2017.1316461PubMedGoogle ScholarCrossref
9.
Salaffi  F, Carotti  M, Guglielmi  G, Passarini  G, Grassi  W.  The crowned dens syndrome as a cause of neck pain.  Clin Exp Rheumatol. 2008;26(6):1040-1046.PubMedGoogle Scholar
10.
Zhang  W, Doherty  M, Pascual  E,  et al.  EULAR recommendations for calcium pyrophosphate deposition.  Ann Rheum Dis. 2011;70(4):571-575. doi:10.1136/ard.2010.139360PubMedGoogle ScholarCrossref
If you are not a JN Learning subscriber, you can either:
Subscribe to JN Learning for one year
Buy this activity
jn-learning_Modal_LoginSubscribe_Purchase
If you are not a JN Learning subscriber, you can either:
Subscribe to JN Learning for one year
Buy this activity
jn-learning_Modal_LoginSubscribe_Purchase
With a personal account, you can:
  • Access free activities and track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
Education Center Collection Sign In Modal Right

Name Your Search

Save Search
With a personal account, you can:
  • Track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
jn-learning_Modal_SaveSearch_NoAccess_Purchase

Lookup An Activity

or

My Saved Searches

You currently have no searches saved.

With a personal account, you can:
  • Access free activities and track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
Education Center Collection Sign In Modal Right
Topics
State Requirements