In this case report we discuss a case of IgG4 related cranial pachymeningitis with isolated extraocular muscle paralysis in a middle aged woman. MRI Brain showed thickened and enhancing pachymeninges and superior recti muscles, with raised serum IgG4 levels. A diagnosis of “possible” IgG4 Related pachymeningitis was considered, and was treated with steroids. She responded well over a period of two months. At the end of two months, the clinical response was good and serum IgG4 levels became normal. IgG4 RD may be considered as a possible differential in all cases of idiopathic pachymeningitis in view of excellent therapeutic response to steroids.
Keywords: IgG4 related cranial pachymeningitis, extraocular muscle
paralysis, corticosteroids