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Prion diseases.

James W Ironside¹, Diane L Ritchie¹, Mark W Head¹

¹National CJD Research and Surveillance Unit, Centre for Clinical Brain Sciences, School of Clinical Sciences, University of Edinburgh, Western General Hospital, Edinburgh, United Kingdom.

Handbook of Clinical Neurology

|October 9, 2017

Summary

This summary is machine-generated.

Human prion diseases are rare, fatal neurodegenerative conditions caused by prion protein misfolding. Diagnosis requires a multidisciplinary approach due to varied clinical presentations.

Keywords:

Creutzfeldt–Jakob disease Gerstmann–Sträussler–Scheinker disease biochemistry fatal familial insomnia neuropathology prion protein variably protease-sensitive prionopathy variant Creuztfeldt–Jakob disease

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Area of Science:

Neurology
Neurodegenerative Diseases
Prion Biology

Background:

Human prion diseases are a group of rare, fatal neurodegenerative disorders.
These diseases include Creutzfeldt-Jakob disease, variably protease-sensitive prionopathy, Gerstmann-Sträussler-Scheinker disease, fatal familial insomnia, and kuru.
Pathophysiology is linked to conformational changes in the prion protein.

Purpose of the Study:

To provide an overview of human prion diseases.
To highlight the diagnostic challenges posed by their heterogeneous phenotypes.
To emphasize the importance of a combined diagnostic approach.

Main Methods:

Review of existing literature and clinical data.
Analysis of genetic, neuropathologic, and biochemical findings.
Integration of clinical observations with laboratory diagnostics.

Main Results:

Human prion diseases manifest in sporadic, genetic, iatrogenic, and zoonotic forms.
Sporadic Creutzfeldt-Jakob disease is the most common form.
Genetic forms are linked to mutations in the prion protein gene (PRNP).
Transmissible forms (iatrogenic and variant Creutzfeldt-Jakob disease) have public health implications.

Conclusions:

Accurate diagnosis of human prion diseases necessitates a comprehensive strategy integrating clinical, genetic, neuropathologic, and biochemical data.
Understanding prion diseases offers insights into broader neurodegenerative mechanisms, including those in Parkinson and Alzheimer diseases.
Continued research is crucial for improving diagnosis and management of these complex conditions.