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Abstract
Recurrent Aseptic Basal Meningitis as the First Clinical Manifestation of a Sjogren Syndrome in a Patient with an Overlap Syndrome with Familial Mediterranean Fever
Highlights
Sjogren Syndrome (SjS) is a chronic autoimmune disease which affects exocrine glands by inflammatory infiltration and secondary dysfunction
We present a patient for the first time with an overlap syndrome of SjS and Familial Mediterranean Fever (FMF) suffering from recurrent aseptic meningitis which precedes the diagnosis of SjS and shows imaging signs of a basal meningitis
In a basal and aseptic meningitis SjS should be considered in the differential diagnosis
Summary
Sjogren Syndrome (SjS) is a chronic autoimmune disease which affects exocrine glands by inflammatory infiltration and secondary dysfunction. We present a patient for the first time with an overlap syndrome of SjS and FMF suffering from recurrent aseptic meningitis which precedes the diagnosis of SjS and shows imaging signs of a basal meningitis. Case: A female patient suffered from biopsy-proven SjS and genetically diagnosed FMF. Over two years she presented with recurrent attacks of aseptic meningitis, while lacking symptoms of polyserositis indicating FMF not being causative. These attacks preceded the diagnosis of SjS. The CNS involvement includes myelo-pathy, cortical lesions as multiple sclerosis-like syndrome, meningoencephalitis and aseptic meningitis [6]. Meningitis persisted during colchicine therapy but terminated after initiating immunosuppressive treatment
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