A Case of Progressive Multifocal Leukoencephalopathy in Acquired Immune Deficiency Syndrome Initially Presented with Early Onset Dementia

Pyeong Kang Park; Jung Geun Oh; Seong-Ho Koh; Kyu-Yong Lee; Young Joo Lee; Hojin Choi

doi:10.12779/dnd.2014.13.1.20

Journal List > Dement Neurocogn Disord > v.13(1) > 1120723

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Park, Oh, Koh, Lee, Lee, and Choi: A Case of Progressive Multifocal Leukoencephalopathy in Acquired Immune Deficiency Syndrome Initially Presented with Early Onset Dementia

Case Report

Dementia and Neurocognitive Disorders 2014; 13(1): 20-23.

Published online: 31 March 2014

DOI: https://doi.org/10.12779/dnd.2014.13.1.20

A Case of Progressive Multifocal Leukoencephalopathy in Acquired Immune Deficiency Syndrome Initially Presented with Early Onset Dementia

Pyeong Kang Park, M.D., Jung Geun Oh, M.D., Seong-Ho Koh, M.D., Kyu-Yong Lee, M.D., Young Joo Lee, M.D., Hojin Choi, M.D.

Department of Neurology, Hanyang University College of Medicine, Seoul, Korea.

Address for correspondence: Hojin Choi, M.D. Department of Neurology, Hanyang University College of Medicine Guri Hospital, 153 Gyeongchun-ro, Guri, 471-701, Korea. Tel: +82-31-560-2260, Fax: +82-31-560-2261, aescu@hanmail.net

Received 4 February 2014 Revised 26 March 2014 Accepted 26 March 2014

(open-access):

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Progressive multifocal leukoencephalopathy (PML) is a very rare and often fatal demyelinating disease of central nervous system (CNS), which mostly occurs in patients with immunosuppression such as acquired immunodeficiency syndrome (AIDS) patients, transplant patients and patients receiving chemotherapy. PML usually manifests with acute or subacute neurologic deficit. and its late diagnosis may lead death or significant permanent disability. We report a 33-year old man diagnosed with PML in AIDS, who initially presented with gradual onset of dementia. Most symptoms of PML were progressed rapidly for several months, and characterized by focal neurological symptoms. On the other hand, we were experienced in patients without focal neurological symptoms and ongoing overall cognitive decline slowly. Patients with immunosuppression can be presented in a variety of neurological symptoms, detailed examinations for cognitive functions were needed in early stage of the disease.

Keywords: Progressive multifocal leukoencephalopathy, Acquired immune deficiency syndrome, Early onset dementia

Figures and Tables

Fig. 1

There is no mass effect. No enhancement was present on contrast-enhanced T1-weighted image (A), with some peripheral restricted on axial diffusion weighted images (B). A part of cortical gray matter was also involved. Brain MRI showed multiple patchy T2 white matter hyperintensities in the left fronto-parieto-temporal subcortical and bilateral periventricular white matter area (C, D).

Table 1

Neuropsychological test profile of the patient

Barthel ADL, Seoul-Instrument Activities of Daily Living; K-MMSE, Korean-Mini-mental State Examination; CDR, Clinical Dementia Rating; K-BNT, Korean Boston naming test; RCFT, Rey Complex Figure Test; SVLT, Seoul Verbal Learning Test; K-CWST, Korean-Color Word Stroop Test.

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