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Prion diseases are a group of rare neurodegenerative conditions characterised by a high rate of progression and highly heterogeneous phenotypes. Whilst the most common form of prion disease occurs sporadically (sporadic Creutzfeldt–Jakob disease, sCJD ...
Liane S. Canas +16 more
doaj +1 more source
MRI lesion profiles in sporadic Creutzfeldt–Jakob disease
With respect to sporadic Creutzfeldt-Jakob disease (sCJD), six molecular subtypes (MM1, MM2, MV1, MV2, VV1, and VV2) have been described, which vary with respect to age at disease onset, disease duration, early symptoms, and neuropathology. MRI signal alterations were reported to correlate with distinct Creutzfeldt-Jakob disease (CJD) subtypes.
Meissner, B. +17 more
openaire +5 more sources
A novel protective prion protein variant that colocalizes with kuru exposure. [PDF]
BACKGROUND: Kuru is a devastating epidemic prion disease that affected a highly restricted geographic area of the Papua New Guinea highlands; at its peak, it predominantly affected adult women and children of both sexes.
Whittaker, John +26 more
core +1 more source
Transmissible spongiform encephalopathies (TSEs) are fatal neurodegenerative diseases with no cure to this day, and are often associated with the accumulation of amyloid plaques in the brain and other tissues in affected individuals. The emergence of new
Keevil, C.William +4 more
core +1 more source
The physicochemical nature of the infectious agent in prion diseases creates asignificant challenge for decontamination services. It has been shown to be both resistant tostandard methods of decontamination, used to inactivate viruses and bacteria, and ...
Howlin, Robert
core
All clinically-relevant blood components transmit prion disease following a single blood transfusion: a sheep model of vCJD [PDF]
Variant CJD (vCJD) is an incurable, infectious human disease, likely arising from the consumption of BSE-contaminated meat products. Whilst the epidemic appears to be waning, there is much concern that vCJD infection may be perpetuated in humans by the ...
Smith, Antony +58 more
core +1 more source
Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease
Several molecular subtypes of sporadic Creutzfeldt-Jakob disease have been identified and electroencephalogram and cerebrospinal fluid biomarkers have been reported to support clinical diagnosis but with variable utility according to subtype.
Taratuto, A. +26 more
core
Human prion diseases can occur as an idiopathic disorder (sporadic Creutzfeldt–Jakob disease) or can be acquired, as is the case for variant Creutzfeldt–Jakob disease.
MacKenzie, Jan +11 more
core +1 more source
Genome-wide association study of behavioural and psychiatric features in human prion disease.
Prion diseases are rare neurodegenerative conditions causing highly variable clinical syndromes, which often include prominent neuropsychiatric symptoms.
Carswell, C +9 more
core
Cortical restricted diffusion as the predominant MRI finding in sporadic Creutzfeldt-Jakob disease
Creutzfeldt-Jakob disease is a rare and fatal neurodegenerative disorder with MR findings predominantly limited to the grey matter of the cortex and the basal ganglia.
Ronald J Sattenberg +4 more
core +1 more source

