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Prion diseases are rare in children. Three types are known: kuru, variant Creutzfeldt-Jakob disease (CJD), and iatrogenic CJD. All three affect children and young adults, and are transmitted by infectious contamination. Kuru was the result of ritual funeral practices similar to cannibalism; variant CJD affects young people who have eaten meat from cows
Felix F, Benninger, Israel, Steiner
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Creutzfeldt-Jakob disease: A case report. [PDF]
Creutzfeldt–Jakob Disease (CJD) is a rare, fatal neurodegenerative disorder that is caused by prion proteins. Patients often present with rapidly progressive dementia, ataxia, myoclonus, memory impairment, visual problems, and changes in personality.
Butt FR, Dhivagaran T, Naqvi S.
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Developing neuropalliative care for sporadic Creutzfeldt-Jakob Disease
We aimed to identify targets for neuropalliative care interventions in sporadic Creutzfeldt-Jakob disease by examining characteristics of patients and sources of distress and support among former caregivers.
Krista L. Harrison +6 more
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Mitochondrial dysfunction has been implicated in multiple neurodegenerative diseases but remains largely unexplored in Creutzfeldt-Jakob disease. Here, we characterize the mitochondrial respiratory chain at the individual neuron level in the MM1 and VV2 ...
Irene H. Flønes +7 more
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Creutzfeldt-Jakob disease [PDF]
This label is applied to a group of patients whose neurological and psychiatric symptoms are somewhat varied and whose brains show pathological changes which are basically similar, although their distribution and extent in the nervous system may vary (Siedler and Malamud, 1963). In the early 19203 A.
L J, Burger, A J, Rowan, E S, Goldensohn
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In the present manuscript, we report the clinical presentation and challenging diagnostic work-up of a sporadic Creutzfeldt–Jakob disease patient with confirmed VV1 subtype and heterozygous 1-octapeptide repeat deletion in the prion protein gene.
Aušrinė Areškevičiūtė +4 more
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Characterization of Prion Disease Associated with a Two-Octapeptide Repeat Insertion
Genetic prion disease accounts for 10–15% of prion disease. While insertion of four or more octapeptide repeats are clearly pathogenic, smaller repeat insertions have an unclear pathogenicity.
Nicholas Brennecke +13 more
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Creutzfeldt–Jakob disease [PDF]
AbstractCreutzfeldt–Jakob disease (CJD) is a progressive, degenerative, and fatal disease of the central nervous system. It is caused by abnormal accumulation of prion proteins and is characterized mainly by progressive dementia, myoclonus, and cerebellar, pyramidal, and extrapyramidal findings. Psychiatric symptoms may also accompany CJD and are often
Ali Görkem, Gençer +4 more
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Variant Creutzfeldt–Jakob disease [PDF]
Summary. Variant Creutzfeldt–Jakob disease (CJD) is an emerging form of human prion disease caused by oral exposure to the bovine spongiform encephalopathy agent. Most cases have occurred in the UK, but smaller numbers of cases have been identified in 10 other countries worldwide.
H J T, Ward +3 more
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Creutzfeldt-Jakob disease. [PDF]
Summary The laboratory transmission to animals of an apparently degenerative disease of the nervous system, Creutzfeldt-Jakob disease (CJD), is now well established. Important questions arising from this observation are the possibility of natural transmission or infectivity and the existence of other similarly transmissible diseases ...
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