title: [Orbital apex syndrome due to relapse during steroid tapering in a patient with MPO-ANCA-positive IgG4-related hypertrophic pachymeningitis].
abstract: A 75-year-old man developed hearing loss and hoarseness; 5 months later, he suffered from headache and loss of appetite. A blood test showed an inflammatory reaction, a high level of serum IgG4 (254.0 mg/dl), and positive reaction for MPO-ANCA. Gadolinium enhanced T1 weighted head magnetic resonance imaging (MRI) revealed dural thickening with marked enhancement. Infiltration of lymphocytes and anti-IgG4-positive plasma cells were detected in the dura mater by meningeal biopsy; thus, he was diagnosed with MPO-ANCA-positive IgG4-related hypertrophic pachymeningitis. His clinical manifestations, and serologic and MRI findings improved with steroid treatment; however, they recurred during steroid tapering and he presented with right orbital apex syndrome. We then added an immunosuppressive drug to his regimen. It was difficult to reduce the symptoms of this case, with oral steroid monotherapy, and its combination with an immunosuppressive drug was necessary.
authors.affiliation: Department of Neurology, Dokkyo Medical University Koshigaya Hospital.
authors.forename: Masaoki
authors.initials: M
authors.lastname: Iwanami
authors.forename: Tomohiro
authors.initials: T
authors.lastname: Ogawa
authors.forename: Yoshinori
authors.initials: Y
authors.lastname: Tanaka
authors.forename: Akio
authors.initials: A
authors.lastname: Hyoudo
authors.forename: Yoshihiko
authors.initials: Y
authors.lastname: Ueda
authors.forename: Tomoyuki
authors.initials: T
authors.lastname: Miyamoto
mesh_terms: D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D006801:Humans; D006984:Hypertrophy; D007074:Immunoglobulin G / Q000276:immunology*; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D008297:Male; D008581:Meningitis / Q000150:complications / Q000188:drug therapy* / Q000276:immunology; D008775:Methylprednisolone / Q000008:administration & dosage*; D009916:Orbital Diseases / Q000209:etiology*; D009195:Peroxidase / Q000276:immunology; D012008:Recurrence; D016559:Tacrolimus / Q000008:administration & dosage
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D009195:Peroxidase; D016559:Tacrolimus; D008775:Methylprednisolone