Neurological manifestations of the Mendelian-inherited autoinflammatory syndromes
نویسندگان
چکیده
منابع مشابه
Diversity in clinical manifestations of autoinflammatory syndromes
Results Fifty six patients were included: 17 CAPS, 4 TRAPS, 5 HIDS, 18 FMF, 6 CRMO, 2 SAPHO and 4 Behcet. The median follow-up period was 2 years (0-14 years). The male/female ratio was 20/36. The median age was 2.5 years at disease onset and 4 years at diagnosis. Family history was positive in 34% of patients. Clinical manifestations included fever (79%), mucocutaneous (61%), musculoskeletal (...
متن کاملNeurological manifestations in autoinflammatory syndromes: a series of 131 patients from our neuroimmunology departement
Introduction Cryopyrin-associated periodic syndrome (CAPS), familial mediterranean fever (FMF) and tumor necrosis factor receptor-associated periodic syndromes (TRAPS) are rare systemic, monogenetic inherited autoinflammatory diseases. The clinical significance of low penetrance mutations in TRAPS and CAPS as well as heterozygosity in FMF is still under debate. The frequency and clinical patter...
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There has been an expansion of the autoinflammatory syndromes due to the discovery of new diseases related to mutations in genes regulating the innate immune system and the knowledge gained from these diseases as applied to more common nongenetic inflammatory conditions. Autoinflammatory syndromes are characterized by unprovoked (or triggered by minor events) recurrent episodes of systemic infl...
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ژورنال
عنوان ژورنال: Developmental Medicine & Child Neurology
سال: 2009
ISSN: 0012-1622,1469-8749
DOI: 10.1111/j.1469-8749.2009.03336.x