title: [Hypertrophic pachymeningitis secondary to IgG4-related disease: case report and review of the literature].
abstract: BACKGROUND
  Hypertrophic pachymeningitis is an infrequent disorder that produces focal or diffuse thickening of the dura mater. It can be idiopathic or secondary to infectious, autoimmune or neoplastic processes. The recently described 'IgG4-related disease' could be the cause of many cases considered cryptogenic.
  
  
  METHODS
  A 54-year-old woman, with a history of bronchial asthma, presented with headache, dizziness and hearing loss on her left ear. The brain MRI study with gadolinium showed enhancement and thickening of the dura mater, extending from lateral wall of left cavernous sinus and medial temporal lobe to cerebellopontine angle and ipsilateral tentorium. CSF had 10 leukocytes/µL (90% mononuclear), with 1 g/L protein and without glucose consumption. Pathology showed fibrosis and lymphoplasmacytic infiltrate, with 16 IgG4+ plasma cells per high power field. The rest of analytical and microbiological studies were normal or negative. The plasma IgG4 rate was within normal limits. After treatment with steroids there was clinical improvement accompanied by the virtual disappearance of the alterations detected in neuroimaging.
  
  
  CONCLUSIONS
  Hypertrophic pachymeningitis as a manifestation of IgG4-related disease can be diagnosed based on MRI findings if plasma IgG4 is elevated. In doubtful cases we must resort to meningeal biopsy. Corticosteroid therapy is usually effective and it is the first line treatment.
authors.affiliation: Complejo Hospitalario Universitario de Santiago de Compostela, 15706 Santiago de Compostela, Espana.
authors.forename: Emilio
authors.initials: E
authors.lastname: Rodríguez-Castro
authors.forename: Aida
authors.initials: A
authors.lastname: Fernández-Lebrero
authors.forename: Iria A
authors.initials: IA
authors.lastname: López-Dequidt
authors.forename: Xiana
authors.initials: X
authors.lastname: Rodríguez-Osorio
authors.forename: Francisco J
authors.initials: FJ
authors.lastname: López-González
authors.forename: José M
authors.initials: JM
authors.lastname: Suárez-Peñaranda
authors.forename: Manuel
authors.initials: M
authors.lastname: Arias
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D020274:Autoimmune Diseases of the Nervous System / Q000150:complications / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D001402:B-Lymphocytes / Q000276:immunology / Q000473:pathology*; D002426:Cavernous Sinus / Q000473:pathology; D002530:Cerebellopontine Angle / Q000473:pathology; D005072:Evoked Potentials, Auditory; D005260:Female; D005355:Fibrosis; D006801:Humans; D006984:Hypertrophy; D007074:Immunoglobulin G / Q000032:analysis*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008581:Meningitis / Q000150:complications / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D008875:Middle Aged; D059906:Neuroimaging; D012598:Sclerosis; D013116:Spinal Cord / Q000473:pathology; D013702:Temporal Lobe / Q000473:pathology; D014057:Tomography, X-Ray Computed
chemical_list: D000305:Adrenal Cortex Hormones; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents