Crowned dens syndrome: A case series of 72 patients at eight teaching hospitals in Japan

Abstract

Background Crowned dens syndrome (CDS) is characterized by calcification around the odontoid process, accompanied by neck pain. Although CDS is supposedly rare, we regularly diagnose and manage this condition, indicating a perception gap between previous studies and our experience. The purpose of this study was to determine the annual incidence of CDS, time to diagnosis in CDS, as well as the features of CDS. Methods The study design was a retrospective case series study conducted at eight teaching hospitals in Japan. We identified CDS cases from April 2013–March 2015. CDS was diagnosed when patients had acute onset of neck pain and CT showed calcification around the dens and when other diagnoses were unlikely. Results Seventy‐two CDS cases were identified. Mean annual incidence was 4.6 ± 2.3 cases at each hospital. Among those with available data, 57 of 64 had limited rotation (89.1%). The diagnosis of CDS was made in general internal medicine or the emergency medicine department in 61 cases (84.7%). A total of 62 cases (86.1%) were diagnosed within 1 day of presentation, and the median time from initial presentation at the hospital to diagnosis was 0.0 days (25th–75th percentiles, 0.0–1.0). For treatment, NSAIDs were used in 56cases (77.8%) and acetaminophen in 20 cases (27.8%). Conclusion CDS might be more common than has been reported to date. Time to diagnosis of CDS was within 1 day of visiting a teaching hospital. Cervical motion restriction is common in CDS and may be useful in establishing the diagnosis.

Crowned dens syndrome detected by positron emission tomography-computed tomography (PET-CT): a case description

“…CDS is notably marked by a significant limitation in cervical rotation. Nonetheless, in the case of early-stage or atypical symptomatic meningitis, prematurely dismissing the possibility of meningitis as a diagnosis could result in delayed treatment, potentially leading to more severe outcomes, which requires lumbar puncture to rule out meningitis ( 14 ).…”

How to distinguish crowned dens syndrome from acute meningitis?

“…To the Editor, We read the article by Isono et al 1 with great interest. We were impressed not only by the largest number of cases recruited into this collaborative study but also by the detailed data collection under a unified protocol, which successfully delineated clinical features of crowned dens syndrome (CDS).…”

Locally advanced endometrial cancer with multiple immune-related adverse events coinciding with the complete response to radiotherapy after immune checkpoint inhibitor therapy: A case report

“…Serum C-reactive protein level was elevated at 15.6 mg/dL (reference value 0.01–0.30), and CT revealed calcification surrounding the odontoid process. In accordance with the diagnostic criteria ( Isono et al, 2023 ), a diagnosis of acute pseudogout of the neck, known as crowned dens syndrome, was made, and the patient was treated with nonsteroidal anti-inflammatory drugs. Twelve months after the initial visit (4 months after the last dose of pembrolizumab and 1 month after completing radiotherapy), both fatigue and anorexia progressed to grade 3 despite the discontinuation of oral hypoglycemic agents.…”