Clinical features of a recently identified disease: GFAP autoimmunity

Glial fibrillar acidic protein (GFAP) autoimmunity is a recently identified disease at the interface between autoimmune encephalitis and gliopathies. Results of a French cohort study showed that GFAP autoimmunity was generally associated with a favourable outcome, and monophasic course with low risk of relapse [1]. GFAP autoimmunity (autoimmune GFAP astrocytopathy) was first described in 2016 by researchers of the Mayo Clinic (USA) [2]. GFAP is a type III intermediate filament protein with several isoforms and is expressed by astrocytes and ependymocytes in the central nervous system. Auto-antibodies against GFAPα are a biomarker of autoimmune GFAP astrocytopathy. In a French cohort study, all patients from 2 referral centres who tested positive for GFAP antibodies were included (n=46). Clinical, biological, and imaging features were reported, as well as clinical course and outcomes. Median age at onset was 43 years; 65% were male. Other auto...

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Posted on 25 November, 2020