Book contents
- Frontmatter
- Contents
- Preface
- Contributors
- Part I Clinical Syndromes – General
- Part II Clinical Syndromes – Head and Neck
- Part III Clinical Syndromes – Eye
- Part IV Clinical Syndromes – Skin and Lymph Nodes
- Part V Clinical Syndromes – Respiratory Tract
- Part VI Clinical Syndromes – Heart and Blood Vessels
- Part VII Clinical Syndromes – Gastrointestinal Tract, Liver, and Abdomen
- Part VIII Clinical Syndromes – Genitourinary Tract
- Part IX Clinical Syndromes – Musculoskeletal System
- Part X Clinical Syndromes – Neurologic System
- 73 Bacterial Meningitis
- 74 Aseptic Meningitis Syndrome
- 75 Acute Viral Encephalitis
- 76 Intracranial Suppuration
- 77 Spinal Epidural Abscess: Diagnosis and Management
- 78 Myelitis and Peripheral Neuropathy
- 79 Reye's Syndrome
- 80 Progressive Multifocal Leukoencephalopathy
- 81 Cerebrospinal Fluid Shunt Infections
- 82 Prion Diseases
- Part XI The Susceptible Host
- Part XII HIV
- Part XIII Nosocomial Infection
- Part XIV Infections Related to Surgery and Trauma
- Part XV Prevention of Infection
- Part XVI Travel and Recreation
- Part XVII Bioterrorism
- Part XVIII Specific Organisms – Bacteria
- Part XIX Specific Organisms – Spirochetes
- Part XX Specific Organisms – Mycoplasma and Chlamydia
- Part XXI Specific Organisms – Rickettsia, Ehrlichia, and Anaplasma
- Part XXII Specific Organisms – Fungi
- Part XXIII Specific Organisms – Viruses
- Part XXIV Specific Organisms – Parasites
- Part XXV Antimicrobial Therapy – General Considerations
- Index
80 - Progressive Multifocal Leukoencephalopathy
from Part X - Clinical Syndromes – Neurologic System
Published online by Cambridge University Press: 05 March 2013
- Frontmatter
- Contents
- Preface
- Contributors
- Part I Clinical Syndromes – General
- Part II Clinical Syndromes – Head and Neck
- Part III Clinical Syndromes – Eye
- Part IV Clinical Syndromes – Skin and Lymph Nodes
- Part V Clinical Syndromes – Respiratory Tract
- Part VI Clinical Syndromes – Heart and Blood Vessels
- Part VII Clinical Syndromes – Gastrointestinal Tract, Liver, and Abdomen
- Part VIII Clinical Syndromes – Genitourinary Tract
- Part IX Clinical Syndromes – Musculoskeletal System
- Part X Clinical Syndromes – Neurologic System
- 73 Bacterial Meningitis
- 74 Aseptic Meningitis Syndrome
- 75 Acute Viral Encephalitis
- 76 Intracranial Suppuration
- 77 Spinal Epidural Abscess: Diagnosis and Management
- 78 Myelitis and Peripheral Neuropathy
- 79 Reye's Syndrome
- 80 Progressive Multifocal Leukoencephalopathy
- 81 Cerebrospinal Fluid Shunt Infections
- 82 Prion Diseases
- Part XI The Susceptible Host
- Part XII HIV
- Part XIII Nosocomial Infection
- Part XIV Infections Related to Surgery and Trauma
- Part XV Prevention of Infection
- Part XVI Travel and Recreation
- Part XVII Bioterrorism
- Part XVIII Specific Organisms – Bacteria
- Part XIX Specific Organisms – Spirochetes
- Part XX Specific Organisms – Mycoplasma and Chlamydia
- Part XXI Specific Organisms – Rickettsia, Ehrlichia, and Anaplasma
- Part XXII Specific Organisms – Fungi
- Part XXIII Specific Organisms – Viruses
- Part XXIV Specific Organisms – Parasites
- Part XXV Antimicrobial Therapy – General Considerations
- Index
Summary
INTRODUCTION
In their seminal report in 1958, Astrom, Mancall, and Richardson described a progressive neurological syndrome with characteristic neuropathological findings of demyelination, giant astrocytes, and oligodendrocytes with abnormal nuclei. They named the disorder progressive multifocal leukoencephalopathy (PML). The viral etiology of this neurological disease was not determined until later. PML remained a vanishingly rare disorder seen almost exclusively in individuals with underlying immunosuppressive disorders until the advent of the acquired immunodeficiency syndrome (AIDS) pandemic. In developed countries, PML occurs in approximately 1 in 20 of all human immunodeficiency virus (HIV)-infected persons and AIDS is now the predisposing disorder for 90% of all PML cases. More recently, monoclonal antibodies that result in a highly specific alteration of immune function, such as natalizumab, an α-4 integrin inhibitor, and rituximab, a chimeric monoclonal antibody directed against CD20 receptors on B cells, and whose therapeutic applications have become increasingly prevalent, have been associated with PML.
JC VIRUS AND THE PATHOGENESIS OF PML
In 1965, Zu Rhein and Chou identified viral particles in glial nuclei resembling papovavirus. Subsequently, Padgett isolated polyoma virus from PML brain in glial cell cultures. This virus proved to be a double-stranded DNA virus of icosahedral symmetry. It has a simple DNA genome of 5.1 kilobases in a double-stranded, supercoiled form, encapsidated in an icosahedral protein structure measuring 40 nm in diameter. JC virus (JCV) DNA encodes for three capsid (VP1, VP2, and VP3) proteins and three regulatory proteins (agnoprotein, t, and T).
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- Information
- Clinical Infectious Disease , pp. 569 - 574Publisher: Cambridge University PressPrint publication year: 2008