Skip to main content

Advertisement

Log in

Crowned dens syndrome: a neurologist’s perspective

  • Original article
  • Published:
Acta Neurologica Belgica Aims and scope Submit manuscript

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. 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

    Article  CAS  Google Scholar 

  2. 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

    Article  CAS  Google Scholar 

  3. 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

    Article  Google Scholar 

  4. 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

    Article  CAS  Google Scholar 

  5. 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

    Google Scholar 

  6. Fung CS, Tam GK (2016) Crowned dens syndrome: an uncommon cause of cord compression. Hong Kong Med J 22(4):399.e4-5

    PubMed  Google Scholar 

  7. Ledingham D, Cappelen-Smith C, Cordato D (2018) Crowned dens syndrome. Pract Neurol 18(1):57–59

    Article  Google Scholar 

  8. 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

    Article  Google Scholar 

  9. 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

    Article  CAS  Google Scholar 

  10. 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

    Article  CAS  Google Scholar 

  11. Rosenthal AK, Ryan LM (2016) Calcium pyrophosphate deposition disease. N Engl J Med. 374(26):2575–2584

    Article  CAS  Google Scholar 

  12. McCarthy GM, Dunne A (2018) Calcium crystal deposition diseases—beyond gout. Nat Rev Rheumatol. 14(10):592–602

    Article  CAS  Google Scholar 

  13. 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

    Article  Google Scholar 

  14. Steinbach LS (2004) Calcium pyrophosphate dihydrate and calcium hydroxyapatite crystal deposition diseases: imaging perspectives. Radiol Clin North Am. 42(1):185–205 (vii)

    Article  Google Scholar 

  15. Ferrone C, Andracco R, Cimmino MA (2012) Calcium pyrophosphate deposition disease: clinical manifestations. Reumatismo. 63(4):246–252

    Article  CAS  Google Scholar 

  16. Oliva F, Via AG, Maffulli N (2012) Physiopathology of intratendinous calcific deposition. BMC Med. 10:95

    Article  Google Scholar 

  17. Goto S, Umehara J, Aizawa T, Kokubun S (2007) Crowned Dens syndrome. J Bone Joint Surg Am. 89(12):2732–2736

    Article  Google Scholar 

  18. Duizer ML, Hermsen R, Te Boekhorst T, Janssen S (2018) Acute headache and neck pain caused by crowned dens syndrome. Ned Tijdschr Geneeskd 162

  19. 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

    Article  Google Scholar 

  20. 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

    Article  Google Scholar 

  21. Razek AA (2010) Diffusion-weighted magnetic resonance imaging of head and neck. J Comput Assist Tomogr. 34(6):808–815

    Article  Google Scholar 

  22. 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

    Article  Google Scholar 

  23. Shah LM, Salzman KL (2011) Imaging of spinal metastatic disease. Int J Surg Oncol. 2011:769753

    PubMed  PubMed Central  Google Scholar 

  24. Raghavan M, Lazzeri E, Palestro CJ (2018) Imaging of Spondylodiscitis. Semin Nucl Med. 48(2):131–147

    Article  Google Scholar 

  25. 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

    Article  Google Scholar 

Download references

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

Authors

Corresponding author

Correspondence to Lauranne Scheldeman.

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

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

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

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s13760-019-01153-z

Keywords

Navigation