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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.
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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.
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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.
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