Crowned Dens Syndrome Presenting as Altered Mental Status

      A 77-year-old male presented from a rehabilitation facility for acute onset of confusion, significant pain in his neck and extremities, muscle rigidity and subjective fever. Comorbidities included Parkinson disease, Lewy body dementia, anxiety, depression and recent right shoulder surgery complicated by pulmonary embolism, for which he was started on apixaban. On admission, physical exam revealed severely limited range of motion in the neck, diffuse rigidity and weakness in all extremities, diffuse joint pain with movement of extremities and neck, and disorientation. His temperature was 37.6°C and initial investigations revealed a leukocytosis with white blood cell count of 14 thousands/microliter, erythrocyte sedimentation rate of 115 mm/h, C-reactive protein of 308 mg/L, urinalysis with numerous white blood cell counts, many bacteria and leukocyte esterase, with urine culture positive for Klebsiella pneumoniae and Enterococcus faecalis. An acute tick-borne panel was subsequently negative.
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to The American Journal of Medicine
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Aouba 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. 2004; 43: 1508-1512
        • Bouvet JP
        • Le Parc JM
        • Michalski B
        • et al.
        Acute neck pain due to calcifications surrounding the odontoid process: the crowned dens syndrome.
        Arthritis Rheum. 1985; 28: 1417-1420
        • Goto S
        • Umehara J
        • Aizawa T
        • Kokubun S
        Crowned dens syndrome.
        J Bone Joint Surg Am. 2007; 89: 2732-2736.
        • Ledingham D
        • Cappelen-Smith C
        • Cordato D
        Crowned dens syndrome.
        Pract Neurol. 2018; 18: 57-59
        • Salaffi F
        • Carotti M
        • Guglielmi G
        • Passarini G
        • Grassi W
        The crowned dens syndrome as a cause of neck pain: clinical and computed tomography study in patients with calcium pyrophosphate dihydrate deposition disease.
        Clin Exp Rheumatol. 2008; 26: 1040-1046
        • Sato Y
        • Yasuda T
        • Konno S
        • Kuwayama A
        • Komatsu K
        Pseudogout showing meningoencephalitic symptoms: crowned dens syndrome.
        Intern Med. 2004; 43: 865-868
        • Takahashi T
        • Minakata Y
        • Tamura M
        • Takasu T
        • Murakami M
        A rare case of crowned dens syndrome mimicking aseptic meningitis.
        Case Rep Neurol. 2013; 5: 40-46
        • Zhang W
        • Doherty M
        • Bardin T
        • et al.
        European League Against Rheumatism recommendations for calcium pyrophosphate deposition. Part I: terminology and diagnosis.
        Ann Rheum Dis. 2011; 70: 563-570