IgG4-RELATED CRANIO-SPINAL HYPERTROPHIC PACHYMENINGITIS INVOLVING THE INTERNAL AUDITORY CANAL

Y. YANGUE, G. GAMBARACCI, P. FLORIDI, A. FIACCA, A. GUERRIERO, M. GIANSANTI, M. SCIALPI

Article ID: 4231
Vol 30, Issue 3, 2016
DOI: https://doi.org/10.54517/jbrha4231
Received: 6 June 2022; Accepted: 6 June 2022; Available online: 6 June 2022; Issue release: 6 June 2022

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.


Keywords

IgG4;pachymeningitis;intracranial;spine;auditory canal


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