CNS B cell infiltration in tumefactive anti-myelin oligodendrocyte glycoprotein antibody-associated disease

Ryotaro Ikeguchi; Natsuki Kanda; Masaki Kobayashi; Kenta Masui; Masayuki Nitta; Tatsuro Misu; Yoshihiro Muragaki; Takakazu Kawamata; Noriyuki Shibata; Kazuo Kitagawa; Yuko Shimizu

doi:10.1177/20552173241301011

CNS B cell infiltration in tumefactive anti-myelin oligodendrocyte glycoprotein antibody-associated disease

Mult Scler J Exp Transl Clin. 2024 Dec 5;10(4):20552173241301011. doi: 10.1177/20552173241301011. eCollection 2024 Oct-Dec.

Affiliations

¹ Department of Neurology, Tokyo Women's Medical University, Tokyo, Japan.
² Department of Pathology, Tokyo Women's Medical University, Tokyo, Japan.
³ Depertment of Neurosurgery, Tokyo Women's Medical University, Tokyo, Japan.
⁴ Department of Neurology, Tohoku University Graduate School of Medicine, Sendai, Japan.

Abstract

Background: Few studies have examined B cells among patients with anti-myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD), including brain pathology.

Objective: To describe cases of tumefactive MOGAD with B-cell dominant central nervous system (CNS) infiltration.

Methods: In this study, we reviewed three cases with clinical and brain histopathological features with tumefactive MOGAD.

Results: Forty-nine cases of tumefactive brain lesions (TBL) between January 2003 and December 2023 were included; of these, seven had MOGAD. Three underwent a brain biopsy. B-cell dominant CNS infiltration was observed in two cases. In two cases with B-cell dominant CNS infiltration, symptoms included fever, headache, nausea, somnolence, and focal neurological deficits. Cerebrospinal fluid examination revealed both mild pleocytosis and negative oligoclonal IgG bands. Magnetic resonance imaging of the brain revealed large abnormal lesions extending from the basal ganglia to the parietotemporal lobe in both cases. These cases showed a good response to steroids; however, one case relapsed. Brain pathology showed demyelination and perivascular lymphocytic infiltration. One showed small vessel vasculitis. Deposition of the activated complement component was absent or rarely observed. Loss of MOG was observed in two cases.

Conclusion: MOGAD could exhibit B-cell dominant CNS infiltration and small vessel vasculitis. MOGAD should be considered in differential diagnosis of TBL.

Keywords: Anti-myelin oligodendrocyte glycoprotein antibody-associated disease; B cell; brain histopathology; brain tumor; myelin oligodendrocyte glycoprotein; tumefactive brain lesion.