title: Progressive multifocal leukoencephalopathy and CD4+ T-lymphocytopenia in a patient with Sjögren syndrome.
abstract: We report progressive multifocal leukoencephalopathy (PML) and CD4+ T-lymphocytopenia in a 71-year-old man with Sjögren syndrome (SjS). The patient was admitted to our hospital because of progressive dementia and gait disturbance. T2-weighted MR images showed high-intensity lesions in his left frontal white matter thalamus, cerebellum and brainstem. A pathological diagnosis of PML was made by brain biopsy. SjS is frequently accompanied with immunological complications; however, there are few reports on PML in patients with SjS. Recently, isolated CD4+ T-lymphocytopenia is reported to be one of the based immunological conditions associated with the development of PML. In the present case, CD4+ T-lymphocytopenia was also observed on admission, which is also associated with SjS.
authors.affiliation: Department of Neurology and Geriatrics, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu, Gifu 501-1194, Japan. hayashiy@gifu-u.ac.jp
authors.forename: Yuichi
authors.initials: Y
authors.lastname: Hayashi
authors.forename: Akio
authors.initials: A
authors.lastname: Kimura
authors.forename: Shimon
authors.initials: S
authors.lastname: Kato
authors.forename: Akihiro
authors.initials: A
authors.lastname: Koumura
authors.forename: Takeo
authors.initials: T
authors.lastname: Sakurai
authors.forename: Yuji
authors.initials: Y
authors.lastname: Tanaka
authors.forename: Isao
authors.initials: I
authors.lastname: Hozumi
authors.forename: Yuji
authors.initials: Y
authors.lastname: Sunden
authors.forename: Yasuko
authors.initials: Y
authors.lastname: Orba
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Sawa
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Takahashi
authors.forename: Takashi
authors.initials: T
authors.lastname: Inuzuka
mesh_terms: D000368:Aged; D006801:Humans; D007968:Leukoencephalopathy, Progressive Multifocal / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D012859:Sjogren's Syndrome / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D018344:T-Lymphocytopenia, Idiopathic CD4-Positive / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D013997:Time Factors; D015899:Tomography, Emission-Computed, Single-Photon; D054334:Viral Regulatory and Accessory Proteins / Q000378:metabolism; D000086082:Viroporin Proteins
chemical_list: D054334:Viral Regulatory and Accessory Proteins; C042559:agnoprotein, polyomavirus; D000086082:Viroporin Proteins