IgG4 Syndrome Presenting as Leptomeningitis in a Young Woman

IgG4-related pachyleptomeningitis with inflammatory pseudotumor

“…Reported pachymeningitis prevalence rates in IgG4-RD range from 2% to 35% [ 8 ]. Leptomeningitis is even rarer, as only six such cases have been reported [ [1] , [2] , [3] , [4] , [5] , [6] ]. To our knowledge, pachyleptomeningitis in association with IgG4-RD has not been previously reported.…”

“…Among the six previously reported cases of IgG4-related leptomeningitis, three were associated with rheumatoid arthritis [ [2] , [3] , [4] ]. These patients had an elevated rheumatoid factor concentration and anti-cyclic citrullinated peptide antibodies.…”

“…IgG4-related disease (IgG4-RD) is a unique fibroinflammatory disorder with organ system involvement. The neurological manifestations of IgG4-RD are varied, especially leptomeningitis is rare as only six cases have been reported [ [1] , [2] , [3] , [4] , [5] , [6] ]. There is no report of pachyleptomeningitis due to IgG4-RD.…”