Cryopyrin-Associated Periodic Fever Syndrome and the Nervous System
Open Access
- 3 September 2018
- journal article
- review article
- Published by Springer Nature in Current Treatment Options in Neurology
- Vol. 20 (10) , 43
Abstract
The purpose of this review is to highlight the molecular and clinical characteristics of the cryopyrin-associated periodic fever syndrome (CAPS) and its management. CAPS is an autosomal dominantly inherited autoinflammatory disorder associated with mutations in the NLRP3 gene, which ultimately lead to excessive production of interleukin-1β (IL-1β) and systemic inflammation. Typical systemic features include fever, urticarial rash and arthralgia, and ultimately amyloidosis. There are also multiple neurological manifestations including, but not restricted to, headache, sensorineural hearing loss, aseptic meningitis, myalgia and optic nerve involvement.Keywords
Funding Information
- University College London
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