title: IgG4-related cranio-spinal hypertrophic pachymeningitis involving the internal auditory canal.
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.
authors.affiliation: Department of Surgical and Biomedical Sciences, Division of Radiology 2, Perugia University, Santa Maria della Misericordia Hospital, Perugia, Italy.
authors.forename: Y
authors.initials: Y
authors.lastname: Yangue
authors.affiliation: Department of Surgical and Biomedical Sciences, Division of Radiology 2, Perugia University, Santa Maria della Misericordia Hospital, Perugia, Italy.
authors.forename: G
authors.initials: G
authors.lastname: Gambaracci
authors.affiliation: Neuroradiology, Santa Maria della Misericordia Hospital, Perugia, Italy.
authors.forename: P
authors.initials: P
authors.lastname: Floridi
authors.affiliation: Neuroradiology, Santa Maria della Misericordia Hospital, Perugia, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Fiacca
authors.affiliation: Department of Experimental Medicine, Surgical Pathology Unit, School of Medicine, Perugia, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Guerriero
authors.affiliation: Department of Experimental Medicine, Surgical Pathology Unit, School of Medicine, Perugia, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Giansanti
authors.affiliation: Department of Surgical and Biomedical Sciences, Division of Radiology 2, Perugia University, Santa Maria della Misericordia Hospital, Perugia, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Scialpi
mesh_terms: D000293:Adolescent; D001327:Autoimmune Diseases / Q000000981:diagnostic imaging / Q000276:immunology* / Q000473:pathology / Q000628:therapy; D002524:Cerebellar Ataxia / Q000209:etiology; D002574:Cervical Vertebrae / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery; D003131:Combined Modality Therapy; D019299:Decompression, Surgical; D003907:Dexamethasone / Q000627:therapeutic use; D004388:Dura Mater / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery; D004677:Encephalocele / Q000000981:diagnostic imaging / Q000209:etiology; D006801:Humans; D006984:Hypertrophy; D007074:Immunoglobulin G / Q000032:analysis*; D007796:Laminectomy; D008279:Magnetic Resonance Imaging; D008581:Meningitis / Q000000981:diagnostic imaging / Q000276:immunology* / Q000473:pathology / Q000628:therapy; D010579:Petrous Bone / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D010950:Plasma Cells / Q000276:immunology / Q000473:pathology*; D000072078:Positron Emission Tomography Computed Tomography; D012678:Sensation Disorders / Q000209:etiology; D013117:Spinal Cord Compression / Q000000981:diagnostic imaging / Q000209:etiology; D014057:Tomography, X-Ray Computed
chemical_list: D007074:Immunoglobulin G; D003907:Dexamethasone