T h e n e w e ngl a nd j o u r na l o f m e dic i n e
n engl j med 367;23 nejm.org december 6, 2012e34
A n 88-year-old woman presented with a 2-month history of
re-current episodes of acute pain in her neck and knees that were
associated with fevers of 38 to 39.3C. She reported no visual
symptoms, jaw claudica-tion, morning stiffness, or pain in the
upper arms or shoulders. Radiography re-vealed chondrocalcinosis in
the knees and stippled calcifications in the pubic sym-physis.
Computed tomography of the neck showed curvilinear calcifications
of the transverse ligament of the atlas (Panel A, arrows), a linear
calcification (Panel B, arrows), and crown-shaped calcium deposits
surrounding the odontoid process (Panel C, arrowheads). Crowned
dens syndrome is characterized by recurrent neck pain related to
radiodense deposits of hydroxyapatite or calcium pyrophosphate
dihydrate in ligaments around the odontoid process, which create
the appearance of a crown or halo surrounding the odontoid process
on radiographic imaging. Evidence of inflammation (e.g., fever or
elevated levels of C-reactive protein) is typical. A short course
of prednisolone (15 mg per day), followed by administration of
nonsteroidal anti-inflammatory medication, completely alleviated
her symp-toms. Long-term treatment with antiinflammatory agents is
usually unnecessary in patients with this condition.DOI:
10.1056/NEJMicm1100764Copyright 2012 Massachusetts Medical
Society.
Crowned Dens Syndrome
Masami Matsumura, M.D.Satoshi Hara, M.D.
Kanazawa UniversityKanazawa, Japan
A B C
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