Immunoglobulin G4-Related Hypertrophic Pachymeningitis Presenting with Multiple Lower Cranial Nerve Palsies
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Immunoglobulin G4-related disease (IgG4-RD) is a multi-organ condition that infrequently exhibits neurological involvement. Here, we describe a case of IgG4-related hypertrophic pachymeningitis with multiple lower cranial nerve palsies. A 65-year-old man presented with a 5-month history of hoarseness and dysphagia. A neurologic examination revealed multiple lower cranial nerve palsies. Brain magnetic resonance imaging indicated diffuse dural involvement along the left tentorium and temporal fossa. Serum IgG and IgG4 levels were found to be elevated. Ethmoid polyp biopsy revealed chronic inflammation with moderate lymphoplasmacytic and some eosinophilic infiltration. Up to 60 IgG4-positive cells were observed per high-power field, and the measured IgG4/IgG ratio was approximately 10% to 20%. Fluorodeoxyglucose (FDG) positron emission tomography/computed tomography indicated abnormally high FDG uptake in the left posterior fossa, tentorium, and left temporal fossa, with additional involvement of the lung, iliac artery, and renal artery. Based on these findings, we diagnosed the patient with IgG4-related hypertrophic pachymeningitis and successfully treated the condition with steroids and rituximab. IgG4-RD should be differentiated from distinct conditions presenting with multiple cranial nerve palsies accompanied by pachymeningitis.
Abstract
Immunoglobulin G4-related disease (IgG4-RD) is a multi-organ condition that infrequently exhibits neurological involvement. Here, we describe a case of IgG4-related hypertrophic pachymeningitis with multiple lower cranial nerve palsies. A 65-year-old man presented with a 5-month history of hoarseness and dysphagia. A neurologic examination revealed multiple lower cranial nerve palsies. Brain magnetic resonance imaging indicated diffuse dural involvement along the left tentorium and temporal fossa. Serum IgG and IgG4 levels were found to be elevated. Ethmoid polyp biopsy revealed chronic inflammation with moderate lymphoplasmacytic and some eosinophilic infiltration. Up to 60 IgG4-positive cells were observed per high-power field, and the measured IgG4/IgG ratio was approximately 10% to 20%. Fluorodeoxyglucose (FDG) positron emission tomography/computed tomography indicated abnormally high FDG uptake in the left posterior fossa, tentorium, and left temporal fossa, with additional involvement of the lung, iliac artery, and renal artery. Based on these findings, we diagnosed the patient with IgG4-related hypertrophic pachymeningitis and successfully treated the condition with steroids and rituximab. IgG4-RD should be differentiated from distinct conditions presenting with multiple cranial nerve palsies accompanied by pachymeningitis.
참고문헌 (10)
- AbdelRazek MA, "IgG4-related disease of the central and peripheral nervous systems", Lancet Neurol, 17, pp.183~192 (2018)
- Baptista B, "Neurological manifestations of IgG4-related disease", Curr Treat Options Neurol, 19, pp.14~ (2017)
- Della-Torre E, "Immunology of IgG4-related disease", Clin Exp Immunol, 181, pp.191~206 (2015)
- 김성희, "Immunoglobulin G4-Related Hypertrophic Pachymeningitis Mimicking Chiari Malformation", Journal of Clinical Neurology, 12(2), pp.238~240 (2016), 대한신경과학회[DOI]
- Koneczny I, "IgG4 autoantibodies in organ-specific autoimmunopathies: reviewing class switching, antibody-producing cells, and specific immunotherapies", Front Immunol, 13, pp.834342~ (2022)
- Wallace ZS, "The 2019 American College of Rheumatology", European League Against Rheumatism classification criteria for IgG4-related disease, 2020, pp.72, 7~19
- Lanzillotta M, "Advances in the diagnosis and management of IgG4 related disease", BMJ, 369, pp.m1067~ (2020)
- Lu LX, "IgG4-related hypertrophic pachymeningitis: clinical features, diagnostic criteria, and treatment", JAMA Neurol, 71, pp.785~793 (2014)
- Mageau A, "Rituximab for corticosteroid-resistant relapsing IgG4-related intracranial pachymeningitis : report of two cases", Pract Neurol, 18, pp.159~161 (2018)
- Keane JR, "Multiple cranial nerve palsies : analysis of 979 cases", Arch Neurol, 62, pp.1714~1717 (2005)