Hunter Soleymani, MD
Resident
Mayo Clinic Rochester
Rochester, Minnesota, United States
Cara C. Prideaux, MD
Assistant Professor PM&R, PM&R Residency Program Director
Mayo Clinic
Rochester, Minnesota, United States
Crowned Dens Syndrome
Case Description:
A 70-year-old male presented with progressively worsening upper neck pain over the past year without any inciting event. He had significantly limited neck extension and lateral rotation which also induced pain. Additionally, he noted prolonged morning stiffness in his neck. He participated in physical therapy and a home exercise program without much relief. He received mild relief with over-the-counter non-steroidal anti-inflammatory drugs (NSIADs). Interestingly, inflammatory blood markers were unremarkable, but cervical MRI and CT imaging was highly consistent with calcium pyrophosphate deposition disease (CPPD) of the atlantodental articulation, which is known as crowned dens syndrome (CDS). He was subsequently evaluated by rheumatology who recommended abortive oral prednisone tapers over 3-6 days.
Discussions:
CDS is characterized by CPPD that surrounds the top and/or sides of the dens. Clinical features include acute/sub-acute onset of upper neck pain often with elevated inflammatory markers, limited neck rotation, and fever. The pain occasionally radiates to the shoulder, occipital, or temporal regions, and therefore is frequently misdiagnosed. Restricted and painful cervical rotation can be helpful for differentiating CDS from meningitis which is often more painful with neck flexion. Additionally, CDS often lacks the electric jolt phenomena seen with meningitis. Cervical CT is the most useful diagnostic modality and often demonstrates calcium deposits in the transverse ligament of the atlas, atlanto-axial joint, or alar ligament, often in a linear fashion. NSAIDs are the first line pharmacologic treatment and usually leads to symptomatic improvement in days to weeks. For more severe or recurrent cases, steroids have proven to be effective.
Conclusions:
Neck pain is a common condition evaluated by physiatrists. When considering the etiology of neck pain, it is important to maintain a wide differential diagnosis, including crowned dens syndrome, to help guide the most appropriate treatments.
