title: Progressive multifocal leukoencephalopathy in a patient with acquired immunodeficiency syndrome (AIDS) manifesting Gerstmann's syndrome.
abstract: We reported a case of acquired immunodeficiency syndrome (AIDS) via multiple blood transfusions, who manifested progressive multifocal leukoencephalopathy (PML) about 18 months after the development of AIDS. PML initiated with right hemiparesis, dysphasia, and Gerstmann's syndrome and resulted in death within 2 months after the onset. Neuroimaging examinations revealed white matter lesions mainly in the left posterior parietal lobe. The cortical gray matter also showed abnormal signal intensity. Peripheral CD4+ lymphocyte count was 81/microl. Routine cerebrospinal fluid (CSF) examinations were negative. CSF antibodies against herpes simplex virus, varicella-zoster virus, cytomegalovirus, Epstein-Barr virus as well as serum antibody against toxoplasma gondii were negative. Though autopsy or biopsy of the brain was not performed, JC virus genomes were detected in the CSF sample by a polymerase chain reaction, and their sequencing showed unique alterations of the regulatory regions, characteristic to PML-type JC virus.
authors.affiliation: Department of Neurology, Tohoku University School of Medicine, Sendai, Japan.
authors.forename: H
authors.initials: H
authors.lastname: Saito
authors.forename: H
authors.initials: H
authors.lastname: Sakai
authors.forename: K
authors.initials: K
authors.lastname: Fujihara
authors.forename: K
authors.initials: K
authors.lastname: Fujihara
authors.forename: Y
authors.initials: Y
authors.lastname: Itoyama
mesh_terms: D000163:Acquired Immunodeficiency Syndrome / Q000134:cerebrospinal fluid / Q000150:complications* / Q000175:diagnosis; D000328:Adult; D001921:Brain / Q000473:pathology; D018791:CD4 Lymphocyte Count; D002555:Cerebrospinal Fluid / Q000821:virology; D004279:DNA, Viral / Q000032:analysis; D017809:Fatal Outcome; D005862:Gerstmann Syndrome / Q000134:cerebrospinal fluid / Q000175:diagnosis / Q000209:etiology*; D006801:Humans; D007577:JC Virus / Q000235:genetics / Q000302:isolation & purification; D007968:Leukoencephalopathy, Progressive Multifocal / Q000134:cerebrospinal fluid / Q000150:complications* / Q000175:diagnosis; D008279:Magnetic Resonance Imaging; D008297:Male; D016133:Polymerase Chain Reaction; D014057:Tomography, X-Ray Computed
chemical_list: D004279:DNA, Viral