Crowned dens syndrome: an easily overlooked cause of fever and neck stiffness

A Case of Acute Neck Pain: The Crowned Dens Syndrome

“…CPPD deposition disease, also known as pseudogout, is known to have a variety of clinical presentations depending on the area of calcium crystal deposition [9,10]. The first piece of literature highlighting cervical chondrocalcinosis was published in 1980, and subsequently, Bouvet et al christened it "crowned dens syndrome" in 1985 [11,12].…”

“…CDS, a clinical-radiological entity, is fundamentally an attack of pseudogout at the atlantoaxial joint, characterized chiefly by calcification of the cruciform ligament around the odontoid process of the axis vertebra. This causes acute moderate to severe neck pain/stiffness worst at the base of the occiput and fever with raised inflammatory markers (ESR and C-reactive protein) [9][10][11][12]. It most commonly occurs in patients aged 60 years and above who may have other manifestations of CPPD deposition disease, including polyarticular arthritis with or without acute attacks of pseudogout affecting the knee, shoulder or wrist joints [13][14][15].…”

“…Colchicine is also gaining popularity as a therapeutic choice. Patients who cannot tolerate NSAIDs or colchicine are usually treated with low-dose corticosteroids [6,10]. Other risk factors for CPPD disease, such as hypomagnesemia, hyperparathyroidism, hypophosphatemia and hemochromatosis, should also be addressed if present [9,16].…”

“…Rarely, the inflammatory process in cases of chronic CDS can progressively erode the atlantoaxial ligaments and the dens leading to atlantoaxial instability, cervical spinal cord compression and progressive quadriparesis. Misdiagnosis of CDS may result in inappropriate invasive investigations such as lumbar puncture and subsequent treatment with parenteral broad-spectrum antimicrobial therapy or prolonged external neck immobilization [ 1 , 5 , 8 - 10 ].…”

Crowned dens syndrome—case of crystal deposition in cervical spine

“…Crowned dens syndrome (CDS), first described in 1960, usually presents after 60 [ 1 ] with neck and shoulder pain. It is a rare metabolic disorder characterized by calcium pyrophosphate (CPP) crystal deposition around the odontoid process and cruciform ligament of C1 and C2 cervical vertebrae [ 3 ]. Bloods test usually shows raised inflammatory markers [ 3 , 4 , 5 ], swaying physicians to treat for possible infective causes and perform invasive procedures for neck pain instead of focusing on the benign rheumatological cause, which can be easily managed with conservative management.…”