Crowned dens syndrome: a case report followed by an update on differential diagnosis and treatment

Abstract

Introduction Crowned dens syndrome (CDS) is a rare clinical presentation of chondrocalcinosis or calcium pyrophosphate crystal deposition (CPPD) disease, characterized by fever, neck pain and stiffness, associated with increased biological markers of inflammation. Diagnosis is made mainly via imaging, and the best modality remains the magnetic resonance imaging (MRI) of the neck showing calcification and calcium pyrophosphate crystals deposition around the odontoid process (1). The aim of this report is to increase awareness of this rare and ill-known clinical tableau and to review its complications and the latest modalities of its diagnosis and treatment. Clinical case: An 83-year-old female patient presented to the emergency department (ED) of the Middle East Institute of Health (MEIH) with severe acute onset neck stiffness and pain of 10/10 intensity, radiating to the submandibular area and reaching the occipital part of the head, associated with nausea and 2 episodes of chills one day prior to presentation. Patient denied any recent history of trauma or surgery of the head and neck. Physical exam at that time was only remarkable for marked limitation of neck motion, and positive Kernig and Brudzinski signs. No skin changes, facial sinuses tenderness, cervical lymphadenopathies or neck swellings were noted. Vital signs at ED were within normal range except for a low grade fever. Laboratory work-up upon presentation showed hyperleukocytosis (WBC = 14000/mm3) with left shift and a C-reactive protein (CRP) of 189 mg/L (0-5 mg/L). Chest X-Ray only showed degenerative osteoarthritis of the thoracic spinal vertebrae. CT-scan of the brain without IV contrast done urgently showed no acute changes. As Clinical and biological features were suggestive of acute meningitis, cultures were taken including blood, urine and CSF fluid from lumbar puncture. The patient was admitted to the hospital for IV antibiotic treatment with a large spectrum carbapenem and close observation. On next 2 days, the patient continued to develop low grade fever with a fluctuating pattern, and levofloxacin and amikacin shots were added for maximal bacterial coverage. Urine and blood culture yielded positive for Escherichia Coli only resistant to penicillin. CSF fluid analysis and culture was unremarkable. So, the patient continued to receive IV antibiotics for urinary tract infection and bacteremia. And although the cultures were sensitive to the antibiotics given, the patient continued to suffer from fever, headache, neck stiffness with minimal improvement and response to IV acetaminophen. Laboratory markers continued to increase and CRP reached 325. The patient then underwent a series of imaging and laboratory tests to determine the cause of fever, including CT-scan of the chest, abdomen and pelvis with IV contrast, widal, wright, Rose Bengal, stool analysis, stool culture, clostridium difficile toxins, transthoracic and transesophageal cardiac ultrasound that were all negative. Finally, neck MRI was done to rule out spondylodiscitis or a cervical abscess and showed evidence of osteoarthritis on multiple cervical spinal levels, calcification of the odontoid process of C2, and thickening of the synovium and posterior ligaments (Fig C – D). Those results were compatible with a CT-scan of the neck then done and was in favor of a corona of calcification around the odontoid process suggesting a crowned dens syndrome (Fig A – B). The patient was put on colchicine therapy, and started to improve slowly, then corticosteroids were added and yielded a faster improvement. The C-reactive protein finally started to drop until reaching 23 mg/L upon the discharge. The patient was finally discharged on NSAIDs and colchicine. And after 1 month, the patient was seen in an outpatient setting where she reported dramatic improvement of her symptoms.