JMSNI Journal of Multiple Sclerosis and NeuroimmunologyJ Mult Scler Neuroimmunol (Seoul) 2733-8290 2951-2972 Korean Society of Neuroimmunology 10.59578/jmsni.2024.15.1.40 jmsni-2024-15-1-40 Neuroimage 대뇌 실질을 침범한 IgG4연관비후경수막염 An Immunoglobulin G4-Related Hypertrophic Pachymeningitis Involving Cerebral Parenchyma KimKi Hoon 김기훈 MD a b KimSu-Hyun 김수현 MD PhD a HyunJae-Won 현재원 MD PhD a KimHo Jin 김호진 MD PhD a Department of Neurology, Research Institute and Hospital of National Cancer Center, Goyang, Korea 국립암센터 신경과 Department of Neurology, Inje University Sanggye Paik Hospital, Seoul, Korea 인제대학교 상계백병원 신경과 Address for correspondence: Ho Jin Kim, MD, PhD Department of Neurology, Research Institute and Hospital of National Cancer Center, 323 Ilsan-ro, Ilsandong-gu, Goyang 10408, Korea Tel: +82-31-920-2438, Fax: +82-31-905-5524 E-mail: hojinkim@ncc.re.kr 6 2024 30 6 2024 15 1 40 41 18 03 2024 20 06 2024 21 06 2024 Copyright © 2024 by Korean Society of Neuroimmunology 2024 This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

특이 과거력 없는 56세 남자가 두통 및 처음 발생한 경련으로 방문하였다. 두통 이외에 다른 신경학적 이상 소견은 관찰되지 않았으며, immunoglobulin G4 (IgG4) 수치가 98 mg/dL (정상범위, 3.9-86.4 mg/dL)로 증가되어 있었다. 뇌 자기공명영상상 왼쪽 두정엽에서 덩이 모양의 결절성(nodular) 경막 비후가 뇌실질 침범과 함께 관찰되었으며, 왼쪽 대뇌반구 전반에 걸쳐 미만성(diffuse) 경막 비후 소견을 보였다(Fig. 1). 정확한 감별진단을 위해 시행한 조직생검에서 다수의 림프구 침윤이 동반된 섬유화와 많은 IgG4 양성형질 세포(95/high power field)를 확인함으로써 IgG4연관비후경수막염으로 진단하였다. 이후, methotrexate 10 mg/week와 경구 스테로이드(prednisolone 10 mg, every other day)를 3년째 투여 중이며, 증상 및 영상학적 소견(매년 추적 검사를 시행) 모두 호전된 상태이다. IgG4연관비후경수막염은 경막 비후를 특징으로 하나, 드물게 뇌실질 침범과 함께 경련이나 신경학적 이상 소견을 유발하므로 이에 대한 고려를 필요로 한다.1,2

Conflict of Interest

Kim KH, and Hyun JW reports no disclosures. Kim SH received a grant from the National Research Foundation of Korea and National Cancer Center; consulted and received honoraria from Bayer Schering Pharma, Merck Serono, Eisai, Teva-Handok, Celltrion, and Roche. Kim HJ received a grant from the National Research Foundation of Korea and research support from AprilBio, Eisai and UCB; received consultancy/speaker fees from Alexion, Altos Biologics, AstraZeneca, Biogen, Daewoong Pharmaceutical, Eisai, GC Pharma, Handok Pharmaceutical, Kaigene, Kolon Life Science, MDimune, Merck Serono, Mitsubishi Tanabe Pharma, Roche, and Sanofi Genzyme; is a co-editor for the Multiple Sclerosis Journal and an associated editor for the Journal of Clinical Neurology.

 REFERENCES LiLF TsePY TsangFC LoRC LuiWM LeungGK IgG4-related hypertrophic pachymeningitis at the falx cerebrii with brain parenchymal invasion: a case report World Neurosurg 2015 84 591.e7 10 KimEH KimSH ChoJM AhnJY ChangJH Immunoglobulin G4-related hypertrophic pachymeningitis involving cerebral parenchyma J Neurosurg 2011 115 1242 1247 Figure

Brain MRI of patient. Axial (A) and coronal (B) T1 gadolinium-enhanced images showed diffuse dural thickening on left hemisphere and nodular enhancing lesions (arrowheads). FLAIR-hyperintense lesions were observed in the left hemisphere, suggesting the involvement of cerebral parenchyma (C, D). FLAIR, fluid-attenuated inversion recovery.