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Progressive Multifocal
Leukoencephalopathy
Ade Wijaya, MD – February 2020
Introduction
 First described in the late 1950s [Astrom et al. 1958].
 The causative virus was first isolated in 1971 [Padgett et al. 1971] and named JC
virus (JCV)
 The hallmark of PML is demyelination of axons caused by the lysis of infected
oligodentrocytes by JCV.
 HIV + with low CD4
JC Virus
 JCV is a polyomavirus, genetically related to BK virus (BKV) and simian virus 40
(SV40), composed of a 5–13 kb doublestranded circular DNA enclosed in a capsid
without a lipoprotein envelope.
 Oral / fecal transmission
 The JCV archetype can infect various cell types, including brain cells [Tominaga
et al. 1992; O’Neill et al. 2003; Newman and Frisque, 1997].
Clinical Features
Radiology
Diagnostic Criteria
Diagnosis
Management
 Currently there is neither a specific prophylaxis for PML, nor an effective anti-JCV
treatment in the setting of PML
 In the HIV+ population, timely treatment with highly effective cART is an effective
way of preventing the onset of acquired immune deficiency syndrome (AIDS) and
with it PML
 In the absence of an effective anti-JCV treatment, PML outcomes depend entirely
on an individual’s capacity to recover immune system function and respond to JCV
Summary
 Demyelination of axons caused by the lysis of infected oligodentrocytes by JCV
 HIV + with low CD4
 Diagnosis: Clinical; Radiology; PCR; Histopathology
 Currently there is neither a specific prophylaxis for PML, nor an effective anti-JCV
treatment in the setting of PML
 Poor prognosis
THANK YOU
References:
Pavlovic D, Patera AC, Nyberg F, Gerber M, Liu M, Progressive Multifocal Leukeoncephalopathy Consortium. Progressive multifocal leukoencephalopathy: current treatment options and future
perspectives. Therapeutic advances in neurological disorders. 2015 Nov;8(6):255-73.
Tavazzi E, White MK, Khalili K. Progressive multifocal leukoencephalopathy: clinical and molecular aspects. Reviews in medical virology. 2012 Jan;22(1):18-32.

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Progressive Multifocal Leukoencephalopathy

  • 2. Introduction  First described in the late 1950s [Astrom et al. 1958].  The causative virus was first isolated in 1971 [Padgett et al. 1971] and named JC virus (JCV)  The hallmark of PML is demyelination of axons caused by the lysis of infected oligodentrocytes by JCV.  HIV + with low CD4
  • 3. JC Virus  JCV is a polyomavirus, genetically related to BK virus (BKV) and simian virus 40 (SV40), composed of a 5–13 kb doublestranded circular DNA enclosed in a capsid without a lipoprotein envelope.  Oral / fecal transmission  The JCV archetype can infect various cell types, including brain cells [Tominaga et al. 1992; O’Neill et al. 2003; Newman and Frisque, 1997].
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  • 10. Management  Currently there is neither a specific prophylaxis for PML, nor an effective anti-JCV treatment in the setting of PML  In the HIV+ population, timely treatment with highly effective cART is an effective way of preventing the onset of acquired immune deficiency syndrome (AIDS) and with it PML  In the absence of an effective anti-JCV treatment, PML outcomes depend entirely on an individual’s capacity to recover immune system function and respond to JCV
  • 11. Summary  Demyelination of axons caused by the lysis of infected oligodentrocytes by JCV  HIV + with low CD4  Diagnosis: Clinical; Radiology; PCR; Histopathology  Currently there is neither a specific prophylaxis for PML, nor an effective anti-JCV treatment in the setting of PML  Poor prognosis
  • 12. THANK YOU References: Pavlovic D, Patera AC, Nyberg F, Gerber M, Liu M, Progressive Multifocal Leukeoncephalopathy Consortium. Progressive multifocal leukoencephalopathy: current treatment options and future perspectives. Therapeutic advances in neurological disorders. 2015 Nov;8(6):255-73. Tavazzi E, White MK, Khalili K. Progressive multifocal leukoencephalopathy: clinical and molecular aspects. Reviews in medical virology. 2012 Jan;22(1):18-32.