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

Dement Neurocogn Disord. 2014 Mar;13(1):20-23. Korean.
Published online Mar 31, 2014.
© 2014 Korean Dementia Association
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. and 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, Email:
Received Feb 04, 2014; Revised Mar 26, 2014; Accepted Mar 26, 2014.

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.


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


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).
Click for larger image


Table 1
Neuropsychological test profile of the patient
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1. Weissert R. Progressive multifocal leukoencephalopathy. J Neuroimmunol 2011;231:73–77.
2. Berger JR, Levy RM, Flomenhoft D, Dobbs M. Predictive factors for prolonged survival in acquired immunodeficiency syndrome-associated progressive multifocal leukoencephalopathy. Ann Neurol 1998;44:341–349.
3. Lee JH, Lee JH, Lee KH. One case of progressive multifocal leukoencephalopathy complicating chronic myelocytic leukemia. J Korean Neurol Assoc 1996;14:282–288.
4. Park BS, Yu IK, Lee BH. Progressive multifocal leukoencephalopathy isolated to posterior fossa in a patient with AIDS: DWI and 1H-MRS features. J Korean Soc Radiol 2010;63:403–407.
5. Ko HY, Kim HK, Lee HY, Shim YS, Kim JY. Progressive multifocal leukoencephalopathy in a patient with systemic lupus erythematosus. J Korean Rheum Assoc 2008;15:159–164.
6. Kang HH, Pakr JH, Lee MJ. Progressive Multifocal Leukoencephalopathy in a Patient With Myasthenia Gravis and Thymoma. J Korean Neurol Assoc 2012;30:312–315.
7. Tan CS, Koralnik IJ. Progressive multifocal leukoencephalopathy and other disorders caused by JC virus: clinical features and pathogenesis. Lancet Neurol 2010;9:425–437.
8. Berger JR, Kaszovitz B, Post MJ, Dickinson G. Progressive Multifocal Leukoencephalopathy Associated with Human Immunodeficiency Virus Infection: a Review of the Literature with a Report of Sixteen Cases. Ann Intern Med 1987;107:78–87.
9. Horger M, Beschorner R, Beck R, Nägele T, Schulze M, Ernemann U, et al. Common and uncommon imaging findings in progressive multifocal leukoencephalopathy (PML) with differential diagnostic considerations. Clin Neurol Neurosurg 2012;114:1123–1130.
10. Naess H, Glad S, Storstein A, Rinaldo CH, Mork SJ, Myhr KM, et al. Progressive multifocal leucoencephalopathy in an immunocompetent patient with favourable outcome. A case report. BMC Neurol 2010;10:32.
11. Lima MA, Bernal-Cano F, Clifford DB, Gandhi RT, Koralnik IJ. Clinical outcome of long-term survivors of progressive multifocal leukoencephalopathy. J Neurol Neurosurg Psychiatry 2010;81:1288–1291.
12. Engsig FN, Hansen AB, Omland LH, Kronborg G, Gerstoft J, Laursen AL, et al. Incidence, Clinical Presentation, and Outcome of Progressive Multifocal Leukoencephalopathy in HIV-Infected Patients during the Highly Active Antiretroviral Therapy Era: a Nationwide Cohort Study. J Infect Dis 2009;199:77–83.