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
82 - Prion Diseases
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
Transmissible spongiform encephalopathies or prion diseases are chronic neurological disorders characterized by long incubation periods; progressive noninflammatory disease of brain and spinal cord; transmissibility within species and, to a limited extent, across species barriers; a failure of any immune response; and a uniformly fatal course. The pathology is characterized by neuronal loss, gliosis, and vacuoles in cytoplasm of neural cells giving the spongiform appearance on microscopic exam. Infectivity copurifies with an isoform of a normal surface glycoprotein expressed primarily in the central nervous system. The function of the normal prion protein is unknown; but the misfolded protein—the prion—induces posttranslational conversion of normal prion protein with a helical structure into the infectious isoform rich in β-pleated sheets. This abnormal protease-resistant protein accumulates in brain leading to disease. To date no nucleic acid has been detected in the transmissible particle.
Prion diseases are recognized in animals (scrapie, bovine spongiform encephalopathy, and chronic wasting disease of deer and elk) and humans (kuru, Creutzfeldt-Jakob disease [CJD], and the variant of CJD related to bovine spongiform encephalopathy). The human forms of disease can be divided into three groups: (1) sporadic CJD represents 85% to 90% of cases, (2) familial CJD due to mutations in the gene coding for prion protein making up 10% of cases, and (3) transmitted CJD due to iatrogenic transmission, cannibalism in the case of kuru, and, recently, transmission of bovine spongiform encephalopathy to humans.
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- Information
- Clinical Infectious Disease , pp. 581 - 584Publisher: Cambridge University PressPrint publication year: 2008