THE CROWN OF MISFORTUNE: A CASE OF AN UNDER-DIAGNOSED RHEUMATOLOGIC RARITY A Malipeddi MD, GP Parthiban MD, A Zboril MD, K Reddy MD Department of Medicine, Baton Rouge General Medical Center, Baton Rouge, LA
INTRODUCTION: Crowned dens syndrome (CDS) is an under-recognized and often misdiagnosed condition that mimics multiple neurologic and rheumatologic diseases. It is an uncommon presentation of Calcium Pyrophosphate Deposition (CPPD) or "Pseudo- Gout" that manifests as acute attacks of neck pain with fever, nuchal rigidity, and elevated inflammatory markers related to radio-dense deposits of CPPD around the odontoid process. CASE: An 83-year-old male presented with a 7-day history of confusion, progressive weakness, severe head, neck, and shoulder pain along with a maximum documented temperature of 103o F. On examination, he was found to be confused and in distress with severe posterior cervical tenderness, mild tenderness to palpation of the trapezius muscle and peri-scapular region, and pain with neck flexion. Swelling, warmth, and tenderness of the right ankle and knee joints were additionally noted. Initial labs revealed elevated erythrocyte sedimentation rate, C-reactive protein, Creatinine, blood urea nitrogen, and normal white blood cell count and serum uric acid. Computed Tomography (CT) report of the head and neck showed degenerative changes of the cervical region. Given the presentation, meningitis was suspected and the patient was initiated on empiric antibiotics. A lumbar puncture was performed and meningitis work-up was negative. With no clinical improvement after receiving antibiotic therapy, Polymyalgia Rheumatica was considered. Antibiotics were discontinued and the patient was started on Prednisone. The patient reported significant clinical improvement after which CPPD was added to the differential. However, synovial fluid analysis revealed no crystal deposition. On further evaluation of imaging with the radiologist, calcification of the transverse ligament around the odontoid process with surrounding swelling was identified on the CT scan. Also noted was chondrocalcinosis within the left wrist and right knee on the x-ray. These radiographic findings in conjunction with the patient’s clinical presentation confirmed the diagnosis of Crowned Dens Syndrome. DISCUSSION: CDS is a rare condition that is often mistaken for Meningitis or Polymyalgia Rheumatica and is fairly amenable to treatment. Hence, in elderly patients with sudden onset neck pain with stiffness, fever, and features of systemic inflammation, CDS should be considered in the differential diagnoses.
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