Abstract
IgG4-related disease is an immune-mediated fibroinflammatory syndrome that can affect nearly any organ system. Neurological involvement is uncommon. However, hypertrophic pachymeningitis, hypophysitis, and peripheral neuropathies have been described. Hypertrophic pachymeningitis may present with diffuse symptoms such as headache, cognitive changes, or seizure, as well as with focal symptoms dependent upon the involved anatomical location. Headache and multiple cranial neuropathies are the most common presenting features. Many patients have evidence of peripheral systemic disease at the time of presentation, and serum IgG4 levels are often elevated. A diagnosis of IgG4-related hypertrophic pachymeningitis is best made by exclusion of alternative diagnoses along with the presence of characteristic clinical, radiographic, and histological features. Biopsy is highly recommended. Immediate treatment is with high-dose corticosteroids to be tapered down over time. Steroid-sparing agents are often needed. Rituximab seems promising, but further research is needed.
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