The crowned dens syndrome (CDS), also known as periodontoid calcium pyrophosphate dehydrate crystal deposition disease, is typified clinically by severe cervical pain, neck stiffness and atlantoaxial synovial calcification which could be misdiagnosed as meningitis, epidural abscess, polymyalgia rheumatica, giant cell arthritis, rheumatoid arthritis, cervical spondylitis or metastatic spinal tumor. Crystalline deposition on cervical vertebrae is less well known disease entity and only a limited number of cases have been reported to date. Authors report a case of CDS and describe the clinical feature.
Firstly, the crowned dens syndrome (CDS) was named by Bouvet et al. in 1985 as it was presented by cervical pain due to deposition of calcium pyrophosphate dehydrate (CPPD) crystals or hydroxylapatite at cervical spine and shown radiologically as crown or halo-like distribution when radio-opaque density appeared at the top and side of the odontoid process
A 49-year-old female visited the outpatient clinic with a 7-day history of acute onset severe posterior neck pain. On the day of symptom onset, she took an acupuncture and chiropractic manipulation of cervical spine which did not turn out to be helpful, and she experienced gradual worsening of pain and marked restriction of neck motion. There was no history of trauma and medical illness. On physical examination, passive cervical spine movements were significantly reduced without any focal neurologic deficit. Range of neck motion was limited to 20 degrees on rotation whereas extension and flexion were slightly diminished. At the end of the range of neck motion, pain was markedly provoked. Patient's vital signs were stable.
Plain radiographs of the cervical spine with open mouth view revealed abnormal radio-opaque lesion on the right side of the odontoid process (
Combination of prednisolone and nonsteroidal anti-inflammatory drug (NSAID) was taken and tapered off within three weeks, which led to resolve symptoms and normalize hsCRP. Neckbrace was put on to relieve motion-provoked pain during hospitalization. The lesion disappeared on plain radiographs followed in one month, which gave us a diagnostic clue to exclude such a condition from degenerative arthritis of the region (
The crowned dens syndrome is a rare disease entity which radiologically shows calcification at cruciform ligament around odontoid process and clinically presents acute cervico-occipital pain, fever, and neck stiffness accompanied by biological inflammatory reaction as mentioned earlier. In our case, clinical manifestations include severe posterior neck pain of acute onset, significant restriction of neck rotation and elevation of serum CRP. So that clinicians could make a confusion with spondyloarthritis around atlantoaxial joint area.
The duration of pain varies from few days to several weeks, the location can be from suboccipital area to inferior posterior neck, and the characteristics of pain are also diverse from mild neck discomfort to sleep-breaking severe pain
CDS can be misdiagnosed as meningitis, epidural abscess, rheumatoid arthritis, polymyalgia rheumatica (PMR), giant cell arthritis (GCA), cervical spondylitis or metastatic spinal tumor
For diagnosis, CT scan focused on C1-2 region is gold standard to detect calcification in transverse, apical and alar ligament. CT is even more helpful for small sized lesion when three-dimensional reconstruction is possible
Surgical procedure is performed rarely to patients who are diagnosed with CDS, but Baysal et al. demonstrates that 1 among 17 cases of CDS patients who progressively presented neurologic symptom and was treated by decompression surgery. And under microscopic examination, CPPD crystal in fibrous tissue was found and chronic inflammatory change was presented
Authors present a case of CDS as a rare cause of posterior neck pain with limitation of neck rotation. We suggest that clinicians should be aware of clinical feature of CDS for avoiding unnecessary lumbar puncture or biopsy, and cutting down hospitalization period.
This work was supported by the Soonchunhyang University Research Fund.
Open mouth view demonstrates radio-opaque lesion beside the odontoid process (arrow) (
Coronal CT scan at C1/2 level demonstrates atlantoaxial synovial calcifications in a crown- or halo-like distribution around the odontoid process (
Sagittal (