Progressive multifocal leukoencephalopathy with immune reconstitution inflammatory syndrome following treatment for granulomatosis with polyangiitis. Issue 3 (28th May 2018)

• Record Type: Journal Article
• Authors: Sasaki, Fuyuko; Oyama, Genko; Fujimaki, Motoki; Yokoyama, Kazumasa; Ogasawara, Michihiro; Nakamichi, Kazuo; Saijo, Masayuki; Takasaki, Yoshinari; Hattori, Nobutaka
• Is Part Of: Neurology and clinical neuroscience. Volume 6:Issue 3(2018:May)
• Page Start: 83
• Page End: 85
• Publication Date: 2018-05-28
• Subjects: imaging -- neurologic manifestations of systemic disease; Neurology -- Periodicals; Neurosciences -- Periodicals; 616.8
• DOI: 10.1111/ncn3.12192
• Languages: English
• ISSNs: 2049-4173

Abstract

Abstract: 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.