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Progressive multifocal leukoencephalopathy with immune reconstitution inflammatory syndrome following treatment for granulomatosis with polyangiitis
Author(s) -
Sasaki Fuyuko,
Oyama Genko,
Fujimaki Motoki,
Yokoyama Kazumasa,
Ogasawara Michihiro,
Nakamichi Kazuo,
Saijo Masayuki,
Takasaki Yoshinari,
Hattori Nobutaka
Publication year - 2018
Publication title -
neurology and clinical neuroscience
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.125
0ISSN - 2049-4173
DOI - 10.1111/ncn3.12192
Subject(s) - medicine , progressive multifocal leukoencephalopathy , immune reconstitution inflammatory syndrome , granulomatosis with polyangiitis , prednisolone , prednisone , magnetic resonance imaging , discontinuation , methotrexate , pathology , vasculitis , dermatology , gastroenterology , immunology , radiology , multiple sclerosis , human immunodeficiency virus (hiv) , disease , viral load , antiretroviral therapy
A 56‐year‐old man with granulomatosis with polyangiitis ( GPA ) developed progressive multifocal leukoencephalopathy ( PML ) after a combined immune therapy with methotrexate and prednisolone. Despite negative conversion of JC virus after cessation of methotrexate treatment and tapering of prednisone, he developed amnestic aphasia, left hemispatial neglect, and left hemiplegia, which was confirmed as immune reconstitution inflammatory syndrome ( IRIS ) based on a rapid expansion of the lesions bilaterally with marginal gadolinium enhancement on brain magnetic resonance imaging. This case suggests that it may be important to be aware of possibility of IRIS , in cases of worsening neurological symptoms despite discontinuation of immunosuppressive treatments for GPA with PML .