Get access

Headache in a Patient With Crowned Dens: Report of a New Case

Authors


  • Conflict of Interest: MV, PPS, AS, and FM have no conflict of interest. Till Sprenger has received no personal compensation. His employer, the University Hospital Basel, has received compensation for his serving on scientific advisory boards or speaking fees from Novartis, ATI, Genzyme, Actelion, Jansen, Teva, Mitsubishi Pharma Europe, and Biogen Idec. These fees were used for funding of research. PJG is on Advisory Boards for Allergan, Colucid, MAP Pharmaceuticals, Merck, Sharpe and Dohme, eNeura, Autonomic Technologies, Inc., Boston Scientific, Electrocore, Eli-Lilly, Medtronic, Linde Gases, Arteaus, AlderBio, and BristolMyerSquibb. He has consulted for Pfizer, Nevrocorp, Lundbeck, Zogenix, Impax, Zosano, and Dr. Reddy, and has been compensated for expert legal testimony. He has grant support from Allergan, Amgen, MAP, and MSD. He has received honoraria for editorial work from Journal Watch Neurology and for developing educational materials and teaching for the American Headache Society.

Abstract

Background

Crowned dens syndrome (CDS) is a clinical-radiological entity characterized by acute attacks of neck pain with fever, rigidity, general signs of inflammation, and calcification of the periodontoid articular structures.

Methods

Case report with 42 months follow-up.

Case description

An 81-year-old man, who had never suffered from headache before July 2010, developed strictly left-sided headaches. The pain was restricted to the left side of the scalp and felt more intense in the frontal area. The intensity was moderate to high with a throbbing quality. The pain had an orthostatic component and was worsened by neck hyperextension and Valsalva maneuvers. Neurological and general examinations were normal, except for a reduced range of motion of the neck. He was prescribed indomethacin orally 25 mg t.i.d. and had a partial response. After a week, he was given a dosage of 50 mg t.i.d. with complete remission of the pain. Brain magnetic resonance imaging was normal, while an magnetic resonance imaging of the cervical spine showed a non-homogeneous mass behind the odontoid process of C2, narrowing the subarachnoid space in C1, stretching the posterior longitudinal ligament, and touching the left vertebral artery. A computed tomography scan showed calcification of the soft tissue around the odontoid process and a thickening of the left C2 root. After 4 months, the indomethacin dosage was reduced step-by-step. Indomethacin was discontinued in March 2012. Since then, the headache has not recurred.

Discussion

We here present the case of a patient with headache and radiological findings of crowned dens. However, the clinical presentation differed from previous CDS cases in the literature in that the pain was unilateral with frontal localization and throbbing quality, as well as an orthostatic component and lack of either fever or inflammatory signs. The differential diagnosis also includes a remitting form of hemicrania continua, presenting with an atypical presentation, with neuroimaging incidental finding of CDS.

Conclusion

This case widens the spectrum of the clinical presentation of crowned dens, a condition that should be kept in mind in cases of unilateral headache in older patients.

Ancillary