A Case of Definite IgG4-Related Pachymeningitis

• DOI: 10.1212/wnl.0b013e3181f73685
• Publication Date: 2010-10-11T20:47:54Z
• Authors: A. Kosakai; D. Ito; S. Yamada; S. Ideta; Y. Ota; N. Suzuki
• Keywords: 03 medical and health sciences; 0302 clinical medicine; Immunoglobulin G; Plasma Cells; Brain; Humans; Female; Meningitis; Middle Aged; Magnetic Resonance Imaging; 3. Good health

Immunoglobulin G (IgG)4-related disease is a recently defined entity that characterized by elevated serum IgG4 levels and abundant infiltration of IgG4-positive plasma cells high-grade sclerosis the affected region.1 This includes autoimmune pancreatitis, Mikulicz disease, pseudotumor lung, tubulointerstitial nephritis, Riedel thyroiditis,2 but only CNS involvement has been reported so far hypophysitis.3

In this report, case intracranial IgG4-related pachymeningitis, involving dura mater, reported. definite pachymeningitis was both serologically histologically proven, whose clinical course with treatment were followed.

### Classification evidence.

This study provides Class IV evidence methylprednisolone followed oral prednisolone effective in improving symptoms signs affecting CNS.

### Case report.

A 54-year-old woman anterior uveitis scleritis left eye, perceptive deafness, hypothyroidism treated steroid levothyroxine. Bilateral relapsed repeatedly, her vision gradually deteriorated. When she 56 years old, had pain, bulging, ptosis, ophthalmoplegia sensory impairment …