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J Biol Regul Homeost Agents ; 30(3): 915-919, 2016.
Article in English | MEDLINE | ID: mdl-27655521

ABSTRACT

Immunoglobulin G4 (IgG4)-related hypertrophic pachymeningitis, which is a focally or diffusely thickened dura mater and lymphoplasmacytic infiltration with increased IgG4 bearing plasma cells, is a rare disease, and cases involving the whole cervical spine are even rarer. Here, we describe a case of probable IgG4-related hypertrophic pachymeningitis involving the whole cervical spine and the auditory canals in an 18-year-old male. The patient, who had a history of paresthesia and had previously experienced weakness, presented with generalized tonic seizures. A decompressive laminectomy on cervical vertebrae was performed as a matter of urgency, removing intradural fibrous material. The patient responded well to treatment and was discharged walking independently, with no strength deficit to any of the 4 limbs, and with normal blood tests.


Subject(s)
Autoimmune Diseases/immunology , Immunoglobulin G/analysis , Meningitis/immunology , Petrous Bone/pathology , Plasma Cells/pathology , Adolescent , Autoimmune Diseases/diagnostic imaging , Autoimmune Diseases/pathology , Autoimmune Diseases/therapy , Cerebellar Ataxia/etiology , Cervical Vertebrae/diagnostic imaging , Cervical Vertebrae/pathology , Cervical Vertebrae/surgery , Combined Modality Therapy , Decompression, Surgical , Dexamethasone/therapeutic use , Dura Mater/diagnostic imaging , Dura Mater/pathology , Dura Mater/surgery , Encephalocele/diagnostic imaging , Encephalocele/etiology , Humans , Hypertrophy , Laminectomy , Magnetic Resonance Imaging , Meningitis/diagnostic imaging , Meningitis/pathology , Meningitis/therapy , Petrous Bone/diagnostic imaging , Petrous Bone/surgery , Plasma Cells/immunology , Positron Emission Tomography Computed Tomography , Sensation Disorders/etiology , Spinal Cord Compression/diagnostic imaging , Spinal Cord Compression/etiology , Tomography, X-Ray Computed
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