Abstract
IgG4-related disease is a unique fibroinflammatory disorder with organ system involvement, which was first described in Japan. It is characterized by high serum IgG4 levels and infiltration of IgG4-positive plasma cells in several organs. IgG4-related disease can involve the central and peripheral nervous systems, resulting in hypertrophic pachymeningitis, orbital diseases, hypophysitis, and peripheral nerve disease. Sufficient pathological findings are important for diagnosing IgG4-related disease and distinguishing it from mimics. The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria and the 2020 Revised Comprehensive Diagnostic Criteria have recently been published. Herein, we describe a current update of the clinicopathological features, approach to diagnosis, and management of IgG4-related neurological diseases.
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