Prion diseases
Updates to Article Attributes
Human prion diseases, characterised by progressive neurological decline, and eventual death include:
- Creutzfeldt-Jakob disease (sporadic, variant, familial and iatrogenic)
new-variant Creutzfeldt-Jakob disease- fatal familial insomnia
- Gerstmann-Straussler-Scheinker disease
- kuru
- variably protease sensitive prionopathy
Pathology
They are all mediated by prions: abnormally folded self catalysing endogenous proteins which accumulate within the nervous system. Diagnosis has been problematic as imaging findings are variable and CSF protein analysis (14(14-3-3 protein) insensitive. Brain biopsy is accurate but is fraught with difficulties as equipment needs to be discarded (prions have been found to survive standard autoclaving procedures) and staff precautions are difficult to implement.
Radiographic features
MRI
DWI changes are the earliest imaging manifestation prior to FLAIR and cerebral volume changes. Typically, cortical, and deep grey matter (caudate, thalamus and putamen) demonstrates increased signal, not confined to vascular territories.
Treatment and prognosis
There is no curative treatment available for any prion disease.
-<a href="/articles/creutzfeldt-jakob-disease">Creutzfeldt-Jakob disease</a> (sporadic, familial and iatrogenic)</li>-<li><a href="/articles/new-variant-creutzfeldt-jakob-disease">new-variant Creutzfeldt-Jakob disease</a></li>- +<a href="/articles/creutzfeldt-jakob-disease">Creutzfeldt-Jakob disease</a> (sporadic, variant, familial and iatrogenic)</li>
-</ul><h4>Pathology</h4><p>They are all mediated by prions: abnormally folded self catalysing endogenous proteins which accumulate within the nervous system. Diagnosis has been problematic as imaging findings are variable and CSF protein analysis (14-3-3) insensitive. Brain biopsy is accurate but is fraught with difficulties as equipment needs to be discarded (prions have been found to survive standard autoclaving procedures) and staff precautions are difficult to implement.</p><h4>Radiographic features</h4><h5>MRI</h5><p>DWI changes are the earliest imaging manifestation prior to FLAIR and cerebral volume changes. Typically, cortical, and deep grey matter (caudate, thalamus and putamen) demonstrates increased signal, not confined to vascular territories.</p>- +</ul><h4>Pathology</h4><p>They are all mediated by prions: abnormally folded self catalysing endogenous proteins which accumulate within the nervous system. Diagnosis has been problematic as imaging findings are variable and CSF protein analysis (<a title="14-3-3 protein" href="/articles/14-3-3-protein-1">14-3-3 protein</a>) insensitive. Brain biopsy is accurate but is fraught with difficulties as equipment needs to be discarded (prions have been found to survive standard autoclaving procedures) and staff precautions are difficult to implement.</p><h4>Radiographic features</h4><h5>MRI</h5><p>DWI changes are the earliest imaging manifestation prior to FLAIR and cerebral volume changes. Typically, cortical, and deep grey matter (caudate, thalamus and putamen) demonstrates increased signal, not confined to vascular territories.</p><h4>Treatment and prognosis</h4><p>There is no curative treatment available for any prion disease.</p>
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