Abstract
Introduction: Crowned Dens Syndrome is an uncommon manifestation of calcium pyrophosphate deposition disease caused by crystal deposition and associated inflammation in the C1 and C2 area of the spine. Crowded Dens Syndrome typically causes posterior neck pain and limitation in range of motion. This report describes a case of this unusual and challenging diagnosis and its importance in the differential diagnosis of neck pain. Case Report: An 80-year-old woman presented as a transfer from another hospital for evaluation for possible epidural abscess. She noted several weeks of progressive posterior cervical neck pain. The pain was worse with passive motion, especially flexion, and she had decreased range of motion in all directions. She had elevated inflammatory markers, and neck computed tomography (CT) scan was non-diagnostic. Broad-spectrum antibiotics for treatment of a possible abscess or meningitis were initiated without improvement. After arrival at our hospital, a dual-energy CT scan concentrated at the C1–C2 area was performed. This imaging revealed multiple calcific deposits surrounding the odontoid with associated soft tissue thickening consistent with Crowned Dens Syndrome due to calcium pyrophosphate deposition disease. She improved with steroid treatment. Conclusion: Crowned Dens Syndrome presents similarly to infectious, malignant, and other inflammatory causes of neck pain. Because specific imaging is often necessary to make the diagnosis, clinical awareness and suspicion are essential for identifying this condition and prescribing the correct therapy.
Highlights
Crowned Dens Syndrome is an uncommon manifestation of calcium pyrophosphate deposition disease caused by crystal deposition and associated inflammation in the C1 and C2 area of the spine
We report a case of subacute neck pain which had initially been treated as a possible epidural abscess
Due to persistent inflammatory neck pain with no known cause, rheumatology was consulted. They recommended a dualenergy computed tomography (CT) of the cervical spine which showed multiple calcific deposits surrounding the odontoid with associated soft tissue thickening (Figure 1) consistent with Crowned Dens Syndrome
Summary
Subacute neck pain with elevated inflammatory markers has a large differential with several lifethreatening considerations including malignancy, infection, and inflammatory causes [1]. She described about two weeks of increasing posterior and lateral neck pain which worsened with any head movement She was unable to find anything that relieved the pain and felt most comfortable with her neck fully flexed. She noted a mild posterior headache in addition to the neck pain She denied fever, blurry vision, photophobia, cough or cold symptoms, dyspnea, abdominal pain, diarrhea, or dysuria. Due to persistent inflammatory neck pain with no known cause, rheumatology was consulted They recommended a dualenergy CT of the cervical spine which showed multiple calcific deposits surrounding the odontoid with associated soft tissue thickening (Figure 1) consistent with Crowned Dens Syndrome. She was admitted for bacteremia, and her neck pain had recurred She improved with a higher dose of prednisone and was discharged to complete a longer steroid taper
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