Crowned Dens Syndrome: A Rare Cause of Sudden Onset Neck Pain
Abstract
Objective: To report a case of Crowned Dens Syndrome (CDS), which is a rare disorder caused by crystal deposition by calcium pyrophosphate dihydrate in the peridontoid soft tissues surrounding the C1 and C2 vertebrae that presents in elderly with sudden onset neck pain, neck stiffness, fever, and elevated inflammatory markers, with periodontal calcification in a halo or crown configuration on radiography considered diagnostic.
Methods: A 64-year-old diabetic and hypertensive female patient presented with a 6-day history of sudden onset posterior neck pain and stiffness. Movements of the cervical spine were equally limited in all directions, causing marked aggravation of pain. There was no focal neurologic loss. Her inflammatory markers were markedly raised.
Results: Based on radiography, she was diagnosed with Crowned Dens syndrome and started on oral prednisolone, paracetamol, and tizanidine along with topical diclofenac. Oral NSAIDs were contraindicated due to her renal insufficiency.
Conclusion: Crowned Dens Syndrome (CDS) is a rare cause of neck pain. Clinicians should consider this syndrome in their differential diagnosis. Timely diagnosis and treatment of CDS will lead to avoidance of unnecessary investigations and medications in such patients, along with a reduction in the length of stay.Keywords
Full Text:
PDFReferences
- Deroux A. Une cervicalgie fébrile [A febrile neck pain]. Rev Med Interne. 2023;44(7):381–2. doi:10.1016/j.revmed.2023.03.003
- Sakai D, Ono R, Ichibayashi R. Physical findings of crowned dens syndrome. Clin Case Rep. 2023;11(1):e6852. doi:10.1002/ccr3.6852
- Awisat A, Rosner I, Rimar D, et al. Crowned dens syndrome, yet another rheumatic disease imposter. Clin Rheumatol. 2020;39(2):571–4. doi:10.1007/s10067-019-04822-9
- Heck A, Nolan N, Rojas-Moreno C. Crowned Dens Syndrome: Calcium Pyrophosphate Deposition Disease Masquerading as Osteomyelitis. J Rheumatol. 2018;45(10):1422–3. doi:10.3899/jrheum.170921
- Zimmer V. Crowned dens syndrome. Clin Case Rep. 2020;8(10):2088–9. doi:10.1002/ccr3.3058
- Bamgboje AO, Mohandas N. Crowned Dens Syndrome Masquerading as Meningitis. Cureus. 2021;13(1):e12678. doi:10.7759/cureus.12678
- Yamada T, Saitoh T, Hozumi H, et al. Crowned dens syndrome. Acute Med Surg. 2015;2(4):273. doi:10.1002/ams2.98
- Oka 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(1):e9–e13. doi:10.1016/j.jemermed.2015.02.005
- Michel J, Bedel JB, Pinson C, Joly LM. Crowned dens syndrome: an uncommon mimic of meningitis in the emergency department. Am J Emerg Med. 2023;63:180.e1–180.e3. doi:10.1016/j.ajem.2022.09.045
- Igami E, Fukae J, Kanazawa K, Fuse A, Nakajima A, Eguchi H, Hattori N, et al. Two rare diseases, acute calcific retropharyngeal tendinitis, and crowned dens syndrome, mimicking meningitis: A case report. Front Neurol. 2022;13:946222. doi:10.3389/fneur.2022.946222
- Goto S, Umehara J, Aizawa T, Kokubun S. Crowned Dens syndrome. J Bone Joint Surg Am. 2007;89(12):2732-6. doi:10.2106/JBJS.F.01322
- Ross JS, Moore KR. Diagnostic Imaging: Spine. . US: Elsevier Health Sciences; 2015.
- Zeng L, Liao H, Xie M, Ren F, Zhang Y, Wang Q. Images in neuroscience: crowned dens syndrome. Neurol India. 2022;70(3):1329–30. doi:10.4103/0028-3886.349617
- Rosenthal AK. Calcium pyrophosphate deposition and crowned dens syndrome. Cleve Clin J Med. 2021;88(4):206–7. doi:10.3949/ccjm.88a.21008
- Sano M, Yamashita S, Aiba T. 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. 2018;28(1):182–7. doi: 10.1080/14397595.2017.1316461.
- Oka A, Okazaki K, Takeno A, Kumanomido S, Kusunoki R, Sato S, et al. Crowned Dens Syndrome: Report of Three Cases and a Review of the Literature. J Emerg Med. 2015;49(1):e9–e13. doi: 10.1016/j.jemermed.2015.02.005.
- Stack J, McCarthy G. Calcium pyrophosphate deposition (CPPD) disease - Treatment options. Best Pract Res Clin Rheumatol. 2021;35(4):101720. doi:10.1016/j.berh.2021. 101720
- Lee GS, Kim RS, Park HK, Chang JC. Crowned dens syndrome: a case report and review of the literature. Korean J Spine. 2014;11(1):15–7. doi:10.14245/kjs.2014.11.1.15
- Koyfman A, Yaffe D. Crowned dens syndrome. A case report. Neuroradiol J. 2014;27(4):495–7. doi:10.15274/NRJ-2014-10056
- Abdelkefi I, Saidenberg-Kermanac’h N, Boissier MC, Semerano L, Sigaux J. Crowned dens syndrome. Joint Bone Spine. 2022;89(1):105288. doi:10.1016/j.jbspin.2021.105288
DOI: https://doi.org/10.15850/ijihs.v11n2.3083
Article Metrics
Abstract view : 453 timesPDF - 139 times
This Journal indexed by
IJIHS is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License
View My Stats