Tohoku J. Exp. Med., 1998, 186 (3)

Progressive Multifocal Leukoencephalopathy in a Patient with Acquired Immunodeficiency Syndrome (AIDS) Manifesting Gerstmann's Syndrome

HIROSHI SAITO, HIDEAKI SAKAI,1 KAZUO FUJIHARA,2 KANAE FUJIHARA2 and YASUTO ITOYAMA2

The Departments of Neurology and 1Internal Medicine, 2National Nishitaga Hospital, Department of Neurology, Tohoku University School of Medicine, Sendai 982-8555

  • 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+ Iymphocyte count was 8l/ml. 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.
    Key words--- AIDS; Gerstmann's syndrome; progressive multifocal leukoencephalopathy; JC virus isolation from CSF; PML-type regulatory regions
    © 1998 Tohoku University Medical Press

    Tohoku J. Exp. Med., 1998, 186, 169-179
    Address for reprints: Hiroshi Saito, M.D., Department of Neurology, National Nishitaga Hospital, 2-11-11 Kagitori-Honcho, Taihaku-ku, Sendai 982-8555, Japan.

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