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Neurological rarities
Crowned dens syndrome
  1. David Ledingham1,
  2. Cecilia Cappelen-Smith1,2,
  3. Dennis Cordato1,2
  1. 1 Department of Neurology and Neurophysiology, Liverpool Hospital, Liverpool, New South Wales, Australia
  2. 2 Ingham Institute for Applied Medical Research, South Western Sydney Clinical School, University of New South Wales, Sydney, New South Wales, Australia
  1. Correspondence to Dr David Ledingham, Department of Neurology and Neurophysiology, Liverpool Hospital, Liverpool, Sydney, New South Wales 1871, Australia; dledingham3{at}gmail.com

Abstract

Crowned dens syndrome is a rare presentation of calcium pyrophosphate deposition disease. It is characterised by severe occipital pain and neck stiffness. Acute presentations are typically accompanied by fever and an inflammatory response and hence can be misdiagnosed as polymyalgia rheumatica or meningitis. Chronic relapsing presentations may be misdiagnosed as cervicogenic neck pain or occipital neuralgia. We present a patient who presented with a chronic relapsing form of crowned dens syndrome and discuss the epidemiology, typical presentation and management of this eminently treatable condition.

  • clinical neurology
  • headache
  • pain

https://doi.org/10.1136/practneurol-2017-001793

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    Footnotes

    • Contributors All authors have contributed equally.

    • Competing interests None declared.

    • Patient consent Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed. This paper was reviewed by Brendan Davies, Staffordshire, UK, and Nick Silver, Liverpool, UK.