1. [Hypertrophic pachymeningitis secondary to IgG4-related disease: case report and review of the literature].
- Author
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Rodríguez-Castro E, Fernández-Lebrero A, López-Dequidt IA, Rodríguez-Osorio X, López-González FJ, Suárez-Peñaranda JM, and Arias M
- Subjects
- Adrenal Cortex Hormones therapeutic use, Autoimmune Diseases of the Nervous System complications, Autoimmune Diseases of the Nervous System diagnosis, Autoimmune Diseases of the Nervous System diagnostic imaging, Autoimmune Diseases of the Nervous System immunology, Autoimmune Diseases of the Nervous System pathology, B-Lymphocytes immunology, Cavernous Sinus pathology, Cerebellopontine Angle pathology, Evoked Potentials, Auditory, Female, Fibrosis, Humans, Hypertrophy, Immunosuppressive Agents therapeutic use, Magnetic Resonance Imaging, Meningitis complications, Meningitis diagnosis, Meningitis diagnostic imaging, Meningitis immunology, Meningitis pathology, Middle Aged, Neuroimaging, Sclerosis, Spinal Cord pathology, Temporal Lobe pathology, Tomography, X-Ray Computed, Autoimmune Diseases of the Nervous System etiology, B-Lymphocytes pathology, Immunoglobulin G analysis, Meningitis etiology
- Abstract
Introduction: 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., Case Report: 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.
- Published
- 2015