Crowned Dens Syndrome

Abstract

An 80-year-old Japanese woman presented to the clinic with a 1-day history of acute fever, severe neck pain, and shoulder girdle stiffness. Her medical history included pseudogout of the left knee, which occurred 2 years prior and lasted 10 days. She also had osteoarthritis of the knees. There was no social history. The physical examination revealed fever of 38.4°C, a blood pressure of 152/82 mm Hg, and pulse of 92 beats per minute. She had limited rotational cervical motion without neurologic deficit. Kernig sign and Brudzinski sign were negative. Active and passive range of motion of the shoulders was normal. Her C-reactive protein (10.2 mg/dL) level and erythrocyte sedimentation rate (83 mm/h) were elevated. Computed tomography of the neck revealed curvilinear calcifications of the transverse ligament of the atlas (Figures 1 and 2). Crowned dens syndrome was diagnosed, which improved quickly after a nonsteroidal anti-inflammatory drug was administered.Figure 2Coronal computed tomography imaging showed linear calcification in the coronal view (arrow).View Large Image Figure ViewerDownload Hi-res image Download (PPT) Crowned dens syndrome is characterized by severe acute or recurrent neck pain, restricted neck motion (particularly in rotation), shoulder girdle stiffness, and fever due to (perhaps “associated with” is a better term, as you can have deposits without symptoms) deposits of hydroxyapatite or calcium pyrophosphate dihydrate in ligaments around the odontoid process.1Oka A. Okazaki K. Takeno A. et al.Crowned dens syndrome: report of three cases and a review of the literature.J Emerg Med. 2015; 49: e9-e13Abstract Full Text Full Text PDF PubMed Scopus (26) Google Scholar Most patients are >70 years of age and female.2Goto S. Umehara J. Aizawa T. Kokubun S. Crowned dens syndrome.J Bone Joint Surg Am. 2007; 89: 2732-2736Crossref PubMed Scopus (74) Google Scholar Chronic renal failure was associated with the risk of pseudogout.3Rho Y.H. Zhu Y. Zhang Y. Reginato A.M. Choi H.K. Risk factors for pseudogout in the general population.Rheumatology (Oxford). 2012; 51: 2070-2074Crossref PubMed Scopus (43) Google Scholar Inflammation is typical, as seen in most cases of crystal-induced arthritis. Computed tomography is the preferred technique to detect crystal deposition, presenting a crown or halo appearance around the odontoid process.4Takahashi T. Minakata Y. Tamura M. Takasu T. Murakami M. A rare case of crowned dens syndrome mimicking aseptic meningitis.Case Rep Neurol. 2013; 5: 40-46Crossref PubMed Scopus (17) Google Scholar Short-term administration of nonsteroidal anti-inflammatory drugs, steroids, or colchicine enables quick resolution.2Goto S. Umehara J. Aizawa T. Kokubun S. Crowned dens syndrome.J Bone Joint Surg Am. 2007; 89: 2732-2736Crossref PubMed Scopus (74) Google Scholar, 5Aouba A. Vuillemin-Bodaghi V. Mutschler C. De Bandt M. Crowned dens syndrome misdiagnosed as polymyalgia rheumatica, giant cell arteritis, meningitis or spondylitis: an analysis of eight cases.Rheumatology (Oxford). 2004; 43: 1508-1512Crossref PubMed Scopus (95) Google Scholar Crowned dens syndrome can resemble conditions such as meningitis, epidural abscess, rheumatoid arthritis, or polymyalgia rheumatica.6Lee G.S. Kim R.S. Park H.K. Chang J.C. Crowned dens syndrome: a case report and review of the literature.Korean J Spine. 2014; 11: 15-17Crossref PubMed Google Scholar This causes misdiagnosis, unnecessary invasive procedures, inappropriate treatment, and prolonged hospitalization.4Takahashi T. Minakata Y. Tamura M. Takasu T. Murakami M. A rare case of crowned dens syndrome mimicking aseptic meningitis.Case Rep Neurol. 2013; 5: 40-46Crossref PubMed Scopus (17) Google Scholar