Crowned Dens Syndrome

AUTHOR:
Suresh S. Kumar, MD

AFFILIATION:
Veterans Affairs Medical Center, West Palm Beach, Florida

CITATION:
Kumar SS. Crowned dens syndrome. Consultant. 2022;62(5):e20-e21. doi:10.25270/con.2021.10.00011

Received May 26, 2021. Accepted June 15, 2021. Published online October 28, 2021.

DISCLOSURES:
The authors report no relevant financial relationships.

DISCLAIMER:
The views expressed in this article are those of the author and do not necessarily reflect the official policy or position of the Department of Veterans Affairs or the US Government.

CORRESPONDENCE:
Suresh S. Kumar, MD, Veterans Affairs Medical Center, 7305 North Military Trail, West Palm Beach, FL 33410 (Suresh.Kumar2@va.gov)

A 69-year-old man with a medical history significant for hypertension and dyslipidemia presented with recent onset of neck pain for about a month. The pain was situated over the right occipital area. No radicular symptoms or injury were reported.

History. The patient reported that the pain had begun suddenly and had no aggravating or relieving factors. He reported no fever, chills, or rash. Pain was present throughout the day and affected his sleep. He started taking over-the-counter ibuprofen, which he said had helped reduce the pain.

He denied a history of gout, pseudogout, or nephrolithiasis. His family history was noncontributory. He is a nonsmoker and consumed 2 alcoholic beverages daily. He has no allergies. Only medications are losartan and pravastatin.

Physical examination. His vital signs were within normal limits. The rest of the examination was unremarkable, except for decreased range of motion in the neck because of the pain and for tenderness over the occipital area. No rash, neurological deficits, or meningeal signs were noted.

Laboratory studies. The patient’s erythrocyte sedimentation rate was normal at 3 mm/hr, and his C-reactive protein level was elevated at 3.7 mg/dL (reference, < 0.2 mg/dL). Results of a complete blood cell count, liver function test, and renal function test were within normal limits.

A computed tomography (CT) scan of the cervical spine was conducted, results of which showed chondrocalcinosis and erosion of the odontoid (Figures 1-3). Erosion of the odontoid is rare but can occur with calcium pyrophosphate dihydrate (CPPD) arthritis.

Figure 1. A computed tomography scan showed chondrocalcinosis.

Figure 2. A computed tomography scan showed erosion of the dens.

Figure 3. A computed tomography scan (sagittal view) showed dens.

Patient outcome. Over-the-counter ibuprofen was stopped, and the patient was initiated on naproxen, 500 mg twice daily. He did well, and his symptoms resolved in 2 weeks.

Discussion. Coined by Jean-Pierre Bouvet and colleagues in 1985¹, crowned dens syndrome is an inflammatory condition resulting from crystal (CPPD or hydroxyapatite) deposition in the cruciform and alar ligaments surrounding the dens.² It typically presents with pain and elevated inflammatory markers. The differential diagnosis includes meningitis, giant cell arteritis, malignancies, and rheumatoid arthritis. The diagnostic test of choice is CT scan of the cervical spine. Erosions of the odontoid can occur with CPPD.^2,3

Most patients with crowned dens syndrome respond well to nonsteroidal anti-inflammatory drugs (NSAIDs). If patients are unable to take NSAIDs or if contraindications for NSAIDs are present, then colchicine or steroids can be prescribed instead.

 References

1. Bouvet JP, le Parc JM, Michalski B, Benlahrache C, Auquier L. Acute neck pain due to calcifications surrounding the odontoid process: the crowned dens syndrome. Arthritis Rheum. 1985;28(12):1417-1420. https://doi.org/10.1002/art.1780281215

2.  Baysal T, Baysal O, Kutlu R, Karaman I, Mizrak B. The crowned dens syndrome: a rare form of calcium pyrophosphate deposition disease. Eur Radiol. 2000;10(6):1003-1005. https://doi.org/10.1007/s003300051052

3. Muthukumar N, Karuppaswamy U. Tumoral calcium pyrophosphate dihydrate deposition disease of the ligamentum flavum. Neurosurgery. 2003;53(1):103-109. https://doi.org/10.1227/01.neu.0000068861.47199.a8