Crowned Dens Syndrome: A Rare Complication of Calcium Pyrophosphate Crystal Deposition Disease

Abstract

Crowned dens syndrome (CDS) is a clinical entity characterized by neck pain associated with fever, headaches, and neck stiffness, along with radiologic evidence of peri-odontoid process calcification in a characteristic "crown" or "halo" distribution. It is likely an underdiagnosed condition and patients can initially be misdiagnosed, leading to costly evaluation and unnecessary treatment interventions. We present the case of a 76-year-old man who presented to the emergency department (ED) with a 3-day history of progressively worsening neck pain that was associated with headaches, malaise, decreased oral intake, chills, and fever. Initial evaluation was significant for the presence of fever, tachycardia, and elevated inflammatory markers. We report a case of CDS attributed to calcium pyrophosphate deposition and review the pertinent literature about the presentation, diagnostic evaluation, and treatment of this rare clinical entity.

Crowned dens syndrome: Die Krone der Kristallarthropathien

“…Die Therapie ist rein symptomatisch und ähnelt der medikamentösen Behandlung der CPPD-Arthropathie. NSAR werden zur Schmerzlinderung und niedrig dosierte Steroide zur Entzündungshemmung eingesetzt [5]. Diese Kombinationstherapie führt in der Regel zu einer raschen Symptomlinderung.…”

“…Diese Kombinationstherapie führt in der Regel zu einer raschen Symptomlinderung. Bei subakutem oder rezidivierendem CDS wird auch der niedrig dosierte Einsatz von Colchicin zur Langzeitbehandlung empfohlen [5].…”