Detecting immunoglobulin G4-related intracranial arteriopathy with magnetic resonance vessel wall imaging: a preliminary experience in two cases

Abstract Background Detecting immunoglobulin G4 (IgG4)-related intracranial arteriopathy, a rare neurovascular complication of IgG4-related disease, is challenging. While magnetic resonance (MR) vessel wall imaging (VWI) can visualize various neurovascular pathologies, its application to this arteri...

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Main Authors: Koki Mitani, Takeshi Funaki, Masahiro Tanji, Hideo Onizawa, Hajime Yoshifuji, Yasutaka Fushimi, Shinya Torimaki, Kazumichi Yoshida, Susumu Miyamoto
Format: Article
Language:English
Published: BMC 2022-12-01
Series:BMC Neurology
Subjects:
Online Access:https://doi.org/10.1186/s12883-022-03010-8
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author Koki Mitani
Takeshi Funaki
Masahiro Tanji
Hideo Onizawa
Hajime Yoshifuji
Yasutaka Fushimi
Shinya Torimaki
Kazumichi Yoshida
Susumu Miyamoto
author_facet Koki Mitani
Takeshi Funaki
Masahiro Tanji
Hideo Onizawa
Hajime Yoshifuji
Yasutaka Fushimi
Shinya Torimaki
Kazumichi Yoshida
Susumu Miyamoto
author_sort Koki Mitani
collection DOAJ
description Abstract Background Detecting immunoglobulin G4 (IgG4)-related intracranial arteriopathy, a rare neurovascular complication of IgG4-related disease, is challenging. While magnetic resonance (MR) vessel wall imaging (VWI) can visualize various neurovascular pathologies, its application to this arteriopathy has not been reported as of this writing. Case presentation A 74-year-old male and a 65-year-old female manifested multiple cranial nerve palsy and neck pain, respectively. Both cases exhibited multiorgan masses with markedly elevated serum IgG4 levels and were clinically diagnosed with IgG4-related disease. Three-dimensional T1-weighted black blood VWI with and without contrast agent identified intracranial vascular lesions characterized as nearly-circumferential mural thickening with homogeneous contrast enhancement in the internal carotid and vertebral arteries; some of the lesions had been unrecognized with screening MR angiography due to expansive remodeling. The former patient underwent corticosteroid therapy, and VWI after treatment revealed decreased mural thickening and enhancement. Conclusion Further studies to elucidate characteristic findings of VWI might contribute to early detection of this treatable pathology.
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spelling doaj.art-45efd347798d4d76b3c025d12aa2fa522022-12-22T04:41:55ZengBMCBMC Neurology1471-23772022-12-012211610.1186/s12883-022-03010-8Detecting immunoglobulin G4-related intracranial arteriopathy with magnetic resonance vessel wall imaging: a preliminary experience in two casesKoki Mitani0Takeshi Funaki1Masahiro Tanji2Hideo Onizawa3Hajime Yoshifuji4Yasutaka Fushimi5Shinya Torimaki6Kazumichi Yoshida7Susumu Miyamoto8Department of Neurosurgery, Kyoto University Graduate School of MedicineDepartment of Neurosurgery, Kyoto University Graduate School of MedicineDepartment of Neurosurgery, Kyoto University Graduate School of MedicineDepartment of Advanced Medicine for Rheumatic Diseases, Graduate School of Medicine, Kyoto UniversityDepartment of Rheumatology and Clinical Immunology, Kyoto University Graduate School of MedicineDepartment of Diagnostic Imaging and Nuclear Medicine, Kyoto University Graduate School of MedicineDepartment of Neurosurgery, Kyoto University Graduate School of MedicineDepartment of Neurosurgery, Kyoto University Graduate School of MedicineDepartment of Neurosurgery, Kyoto University Graduate School of MedicineAbstract Background Detecting immunoglobulin G4 (IgG4)-related intracranial arteriopathy, a rare neurovascular complication of IgG4-related disease, is challenging. While magnetic resonance (MR) vessel wall imaging (VWI) can visualize various neurovascular pathologies, its application to this arteriopathy has not been reported as of this writing. Case presentation A 74-year-old male and a 65-year-old female manifested multiple cranial nerve palsy and neck pain, respectively. Both cases exhibited multiorgan masses with markedly elevated serum IgG4 levels and were clinically diagnosed with IgG4-related disease. Three-dimensional T1-weighted black blood VWI with and without contrast agent identified intracranial vascular lesions characterized as nearly-circumferential mural thickening with homogeneous contrast enhancement in the internal carotid and vertebral arteries; some of the lesions had been unrecognized with screening MR angiography due to expansive remodeling. The former patient underwent corticosteroid therapy, and VWI after treatment revealed decreased mural thickening and enhancement. Conclusion Further studies to elucidate characteristic findings of VWI might contribute to early detection of this treatable pathology.https://doi.org/10.1186/s12883-022-03010-8CNS vasculitisIgG4-related diseaseMagnetic resonance imagingVessel wall imagingDANTECase report
spellingShingle Koki Mitani
Takeshi Funaki
Masahiro Tanji
Hideo Onizawa
Hajime Yoshifuji
Yasutaka Fushimi
Shinya Torimaki
Kazumichi Yoshida
Susumu Miyamoto
Detecting immunoglobulin G4-related intracranial arteriopathy with magnetic resonance vessel wall imaging: a preliminary experience in two cases
BMC Neurology
CNS vasculitis
IgG4-related disease
Magnetic resonance imaging
Vessel wall imaging
DANTE
Case report
title Detecting immunoglobulin G4-related intracranial arteriopathy with magnetic resonance vessel wall imaging: a preliminary experience in two cases
title_full Detecting immunoglobulin G4-related intracranial arteriopathy with magnetic resonance vessel wall imaging: a preliminary experience in two cases
title_fullStr Detecting immunoglobulin G4-related intracranial arteriopathy with magnetic resonance vessel wall imaging: a preliminary experience in two cases
title_full_unstemmed Detecting immunoglobulin G4-related intracranial arteriopathy with magnetic resonance vessel wall imaging: a preliminary experience in two cases
title_short Detecting immunoglobulin G4-related intracranial arteriopathy with magnetic resonance vessel wall imaging: a preliminary experience in two cases
title_sort detecting immunoglobulin g4 related intracranial arteriopathy with magnetic resonance vessel wall imaging a preliminary experience in two cases
topic CNS vasculitis
IgG4-related disease
Magnetic resonance imaging
Vessel wall imaging
DANTE
Case report
url https://doi.org/10.1186/s12883-022-03010-8
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