Crowned Dens Syndrome as a cause of acute neck pain: a Case Report and Review of the Literature

Abstract

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. INTRODUCTIONCrowned dens syndrome (CDS), also known as acute pseudogout of the cervical spine, is a radioclinical entity defined by the radiographic calcifications in a crown-like configuration around the odontoid process, accompanied clinically with acute neck pain in cervico-occipital area, often with neck stiffness, fevers and raised inflammatory markers. 1 This syndrome was first described by Bouvet et al in 1985. 2 It was postulated that the crowned dens deposits could be attributed to calcium pyrophosphate dihydrate (CPPD) crystals as well as hydroxyapatite crystals. 3 The spontaneous duration of these clinical manifestations is very variable, from a few days to several weeks. These crystalline deposits, most often CPPD crystals, can remain asymptomatic or be responsible for chronic neck pains or spinal cord compression. 4 The aim of this report is to highlight CDS as an important differential diagnosis in patients presenting with acute neck pain. CASE REPORTA 79-year-old woman, known case of hypertension, present to the emergency department of our hospital with acute onset of severe posterior neck pain, occipital headache and fever. The patient also had neck stiffness, vertigo, and vomiting. She was admitted to the medical ward and neurological consultation made suspected meningitis. A computed tomography scan of the head was performed, in which serious pathology was excluded. Neurological evaluation was unremarkable, and rheumatologist opinion was sought. The patient admitted long history of neck pain, which was localized, mild, intermittent, and related mainly to activity. However, two weeks before admission, the patient developed severe posterior neck pain which was acute in onset, continuous, radiating into the occipital region, worst at night and associated with vertigo. She experienced gradual worsening of pain and marked restriction of neck

A Case of Acute Neck Pain: The Crowned Dens Syndrome

“…The exact pathogenesis of CDS is not fully understood, but it is thought to involve chondrocytic transformation of fibroblasts in the ligaments at the atlantoaxial joint with eventual calcium pyrophosphate or hydroxyapatite crystal deposition leading to inflammation of the periodontoid ligaments and surrounding tissues [3,6,13]. Acute or subacute presentations can be misdiagnosed as meningitis, polymyalgia rheumatica, cervical discitis, giant cell arteritis, epidural abscess, rheumatoid arthritis, osteomyelitis, retropharyngeal abscess or a metastatic tumor, and chronic relapsing presentations may be misdiagnosed as cervicogenic neck pain or occipital neuralgia.…”

“…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 - 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 - 15 ].…”