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IgG4-RELATED CRANIO-SPINAL HYPERTROPHIC PACHYMENINGITIS INVOLVING THE INTERNAL AUDITORY CANAL
Vol 30, Issue 3, 2016
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.
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Copyright (c) 2016 Y. YANGUE, G. GAMBARACCI, P. FLORIDI, A. FIACCA, A. GUERRIERO, M. GIANSANTI, M. SCIALPI
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Medical Genetics, University of Torino Medical School, Italy

Department of Biomedical, Surgical and Dental Sciences, University of Milan, Italy