Abstract
Crowned dens syndrome is an under-recognized entity that can mimic neurological disease, in particular meningitis or giant-cell arteritis. We present a 48-year-old woman presenting with an inflammatory meningitis-like syndrome with headache and neck stiffness. Lumbar puncture was normal and computed tomography (CT) of the atlantoaxial joint showed abnormal calcifications around the odontoid process, leading to a tentative diagnosis of crowned dens syndrome. In addition, signs of active inflammation in and around the dens were present on cervical MR imaging. Since CDS can mimic meningitis or giant-cell arteritis, neurologists should be aware of this entity. If CDS is suspected, the bone window on the head CT scan can lead to the diagnosis. On the other hand, asymptomatic periodontoid calcifications are common and should not preclude further investigations.
Similar content being viewed by others
References
Bouvet JP, le Parc JM, Michalski B, Benlahrache C, Auquier L (1985) Acute neck pain due to calcifications surrounding the odontoid process: the crowned dens syndrome. Arthritis Rheum 28(12):1417–1420
Aouba A, Vuillemin-Bodaghi V, Mutschler C, De Bandt M (2004) Crowned dens syndrome misdiagnosed as polymyalgia rheumatica, giant cell arteritis, meningitis or spondylitis: an analysis of eight cases. Rheumatology (Oxford) 43(12):1508–1512
Oka A, Okazaki K, Takeno A, Kumanomido S, Kusunoki R, Sato S et al (2015) Crowned dens syndrome: report of three cases and a review of the literature. J Emerg Med 49(1):e9–e13
Godfrin-Valnet M, Godfrin G, Godard J, Prati C, Toussirot E, Michel F et al (2013) Eighteen cases of crowned dens syndrome: presentation and diagnosis. Neurochirurgie 59(3):115–120
Klineberg E, Bui T, Schlenk R, Lieberman I (2014) Retro-odontoid calcium pyrophosphate dehydrate deposition: surgical management and review of the literature. Evid Based Spine Care J 1:63–69
Fung CS, Tam GK (2016) Crowned dens syndrome: an uncommon cause of cord compression. Hong Kong Med J 22(4):399.e4-5
Ledingham D, Cappelen-Smith C, Cordato D (2018) Crowned dens syndrome. Pract Neurol 18(1):57–59
Kobayashi T, Miyakoshi N, Konno N, Ishikawa Y, Noguchi H, Shimada Y (2018) Age-related prevalence of periodontoid calcification and its associations with acute cervical pain. Asian Spine J 12(6):1117–1122
Sano M, Yamashita S, Aiba T (2018) The prevalence of calcification around odontoid process and the incidence of crowned dens syndrome in the neurosurgical ward: a single institution's analysis. Mod Rheumatol 28(1):182–187
Baysal T, Baysal O, Kutlu R, Karaman I, Mizrak B (2000) The crowned dens syndrome: a rare form of calcium pyrophosphate deposition disease. Eur Radiol. 10(6):1003–1005
Rosenthal AK, Ryan LM (2016) Calcium pyrophosphate deposition disease. N Engl J Med. 374(26):2575–2584
McCarthy GM, Dunne A (2018) Calcium crystal deposition diseases—beyond gout. Nat Rev Rheumatol. 14(10):592–602
Viana SL, Fernandes JL, De Araújo Coimbra PP, De Mendonça JL, Freitas FM, De Carvalho Barbosa Viana MA, (2010) The “v” revisited: imaging findings in calcium crystal deposition diseases around the odontoid. J Neuroimaging 20(4):311–323
Steinbach LS (2004) Calcium pyrophosphate dihydrate and calcium hydroxyapatite crystal deposition diseases: imaging perspectives. Radiol Clin North Am. 42(1):185–205 (vii)
Ferrone C, Andracco R, Cimmino MA (2012) Calcium pyrophosphate deposition disease: clinical manifestations. Reumatismo. 63(4):246–252
Oliva F, Via AG, Maffulli N (2012) Physiopathology of intratendinous calcific deposition. BMC Med. 10:95
Goto S, Umehara J, Aizawa T, Kokubun S (2007) Crowned Dens syndrome. J Bone Joint Surg Am. 89(12):2732–2736
Duizer ML, Hermsen R, Te Boekhorst T, Janssen S (2018) Acute headache and neck pain caused by crowned dens syndrome. Ned Tijdschr Geneeskd 162
Zhang H, Jin D, Sun E (2015) The early and late stages of crowned dens syndrome: two case reports. Spine J. 15(10):e65–68
Jimenez-Boj E, Nobauer-Huhmann I, Hanslik-Schnabel B, Dorotka R, Wanivenhaus AH, Kainberger F et al (2007) Bone erosions and bone marrow edema as defined by magnetic resonance imaging reflect true bone marrow inflammation in rheumatoid arthritis. Arthritis Rheum. 56(4):1118–1124
Razek AA (2010) Diffusion-weighted magnetic resonance imaging of head and neck. J Comput Assist Tomogr. 34(6):808–815
Tomita H, Yamashiro T, Ikeda H, Fujikawa A, Kurihara Y, Nakajima Y (2016) Fluid collection in the retropharyngeal space: A wide spectrum of various emergency diseases. Eur J Radiol. 85(7):1247–1256
Shah LM, Salzman KL (2011) Imaging of spinal metastatic disease. Int J Surg Oncol. 2011:769753
Raghavan M, Lazzeri E, Palestro CJ (2018) Imaging of Spondylodiscitis. Semin Nucl Med. 48(2):131–147
Zibis AH, Giannis D, Malizos KN, Kitsioulis P, Arvanitis DL (2013) Acute calcific tendinitis of the longus colli muscle: case report and review of the literature. Eur Spine J. 22(Suppl 3):S434–438
Acknowledgements
We would like to thank radiologists Philip Van Hover M.D. and Ignace Boelaert M.D. for providing (their advice on) the radiological images.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
All authors declare that they have no conflict of interest.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Scheldeman, L., Van Hoydonck, M., Vanheste, R. et al. Crowned dens syndrome: a neurologist’s perspective. Acta Neurol Belg 119, 561–565 (2019). https://doi.org/10.1007/s13760-019-01153-z
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s13760-019-01153-z