Dement Neurocogn Disord.  2014 Mar;13(1):20-23. 10.12779/dnd.2014.13.1.20.

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

• Park PK
• Oh JG
• Koh SH
• Lee KY
• Lee YJ
• Choi H

• KMID: 1973686
• DOI: http://doi.org/10.12779/dnd.2014.13.1.20

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.

Keyword

Progressive multifocal leukoencephalopathy; Acquired immune deficiency syndrome; Early onset dementia

MeSH Terms

Acquired Immunodeficiency Syndrome*
Adult
Central Nervous System
Delayed Diagnosis
Dementia*
Demyelinating Diseases
Drug Therapy
Hand
Humans
Immunosuppression
Leukoencephalopathy, Progressive Multifocal*
Neurologic Manifestations

Figure

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  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).

Reference

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.