Acute pseudogout of the neck—the crowned dens syndrome: 2 case reports and review of the literature

Abstract

Acute pseudogout of the neck, also known as the crowned dens syndrome, is a rare cause of neck pain characterised by crystalline deposition in periodontoid articular tissues. It is typified clinically by severe cervical pain and stiffness, often in conjunction with pyrexia and raised inflammatory markers. As such, it is often misdiagnosed. We report 2 cases of crowned dens syndrome masquerading respectively as meningitis and polymyalgia rheumatica, and review the literature with particular attention to the clinical and radiological aspects of this under-recognised condition.

Crowned Dens Syndrome Occurred in a Patient after Simple Drainage for Chronic Subdural Hematoma: A Case Report

“…However, abnormal calcification at C1/C2 levels does not appear in this disease. 2,9,10) Calcific tendinitis can be excluded from its location of calcification, which is seen on the lateral tendon of longus colli. 9,10) As for vertebral osteomyelitis, it rarely occurs at the upper cervical portion, and MRI shows abnormal intensity at the vertebral bodies.…”

“…2,9,10) Calcific tendinitis can be excluded from its location of calcification, which is seen on the lateral tendon of longus colli. 9,10) As for vertebral osteomyelitis, it rarely occurs at the upper cervical portion, and MRI shows abnormal intensity at the vertebral bodies. 2,9,10) Among the various diagnostic tools, CT scan is the most useful in detecting calcification of CDS at the C1/C2 region.…”

Crowned Dens Syndrome Incidentally Diagnosed After Investigation of Cervical Spine Injury With Incomplete Quadriplegia: A Case Report and Review of the Literature

“…The syndrome is more common in the elderly, particularly women after the age of 60 [6]. Its clinical presentation includes acute neck pain with concomitant neck stiffness and possibly fever, while the inflammatory markers are often elevated [2,3].…”

“…The gold standard for the diagnosis of the CDS is CT scan [6], because usually plain radiographs fail to highlight the periodontoid calcifications [5]. The CT findings persist for about 3 months after the relief of symptoms of the syndrome [6].…”

“…The syndrome may also be accompanied by neck stiffness, fever, raise of inflammatory indices, chronic neck pain, spinal cord compression or be asymptomatic [2][3][4]. As the plain radiographs are often clear [5], CT scan is considered as the "cornerstone" for the diagnosis of syndrome [6], which is usually treated with non-steroid anti-inflammatory drugs, colchicine and corticosteroids [1][2][3][4][7][8][9]. We present a case of a 73-year-old patient who was diagnosed with this rare syndrome, after a fall of a height of approximately 2 m and cervical spine injury.…”