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Year Number of Results
1993 4
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2000 7
2001 4
2002 8
2003 6
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2006 4
2007 6
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168 results

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Page 1
Prion Disease.
Baldwin KJ, Correll CM. Baldwin KJ, et al. Semin Neurol. 2019 Aug;39(4):428-439. doi: 10.1055/s-0039-1687841. Epub 2019 Sep 18. Semin Neurol. 2019. PMID: 31533183 Review.
There are three main groups of prion diseases, termed sporadic (Creutzfeldt-Jakob disease [CJD], sporadic fatal insomnia, and variably protease-sensitive prionopathy), genetic (genetic CJD, fatal familial insomnia, and Gerstmann-Straussler-Schei …
There are three main groups of prion diseases, termed sporadic (Creutzfeldt-Jakob disease [CJD], sporadic fatal insomnia, and …
Prion Diseases.
Geschwind MD. Geschwind MD. Continuum (Minneap Minn). 2015 Dec;21(6 Neuroinfectious Disease):1612-38. doi: 10.1212/CON.0000000000000251. Continuum (Minneap Minn). 2015. PMID: 26633779 Free PMC article. Review.
RECENT FINDINGS: Prion diseases, a group of disorders caused by abnormally shaped proteins called prions, occur in sporadic (Jakob-Creutzfeldt disease), genetic (genetic Jakob-Creutzfeldt disease, Gerstmann-Straussler-Scheinker syndrome, and fatal familial insomn …
RECENT FINDINGS: Prion diseases, a group of disorders caused by abnormally shaped proteins called prions, occur in sporadic (Jakob-Creutzfel …
Oxidative stress and mitochondrial dysfunction-linked neurodegenerative disorders.
Islam MT. Islam MT. Neurol Res. 2017 Jan;39(1):73-82. doi: 10.1080/01616412.2016.1251711. Epub 2016 Nov 3. Neurol Res. 2017. PMID: 27809706 Review.
In addition, oxidative stress causing protein misfold may turn to other NDDs include Creutzfeldt-Jakob disease, Bovine Spongiform Encephalopathy, Kuru, Gerstmann-Straussler-Scheinker syndrome, and Fatal Familial Insomnia. An overview of the oxidative stress a …
In addition, oxidative stress causing protein misfold may turn to other NDDs include Creutzfeldt-Jakob disease, Bovine Spongiform Encephalop …
Prion diseases.
Ironside JW, Ritchie DL, Head MW. Ironside JW, et al. Handb Clin Neurol. 2017;145:393-403. doi: 10.1016/B978-0-12-802395-2.00028-6. Handb Clin Neurol. 2017. PMID: 28987186 Review.
The human prion diseases comprise Creutzfeldt-Jakob disease, variably protease-sensitive prionopathy, Gerstmann-Straussler-Scheinker disease, fatal familial insomnia, and kuru. Each is a uniformly fatal rare neurodegenerative disease in which conformat …
The human prion diseases comprise Creutzfeldt-Jakob disease, variably protease-sensitive prionopathy, Gerstmann-Straussler-Scheinker disease …
The central autonomic network: functional organization, dysfunction, and perspective.
Benarroch EE. Benarroch EE. Mayo Clin Proc. 1993 Oct;68(10):988-1001. doi: 10.1016/s0025-6196(12)62272-1. Mayo Clin Proc. 1993. PMID: 8412366 Review.
Hyperthermia and autonomic hyperactivity occur in patients with head trauma, hydrocephalus, neuroleptic malignant syndrome, and fatal familial insomnia. In the medulla, the nucleus of the tractus solitarius and ventrolateral medulla contain a network of respi …
Hyperthermia and autonomic hyperactivity occur in patients with head trauma, hydrocephalus, neuroleptic malignant syndrome, and fatal …
Prion disease.
Takada LT, Kim MO, Metcalf S, Gala II, Geschwind MD. Takada LT, et al. Handb Clin Neurol. 2018;148:441-464. doi: 10.1016/B978-0-444-64076-5.00029-6. Handb Clin Neurol. 2018. PMID: 29478593 Review.
Although the first PRNP mutations identified, and most since, are PRNP missense, octapeptide repeat insertions, deletion and nonsense mutations have now also been shown to cause gPrD. Based on clinicopathologic features of familial disease, gPrDs historically have been cla …
Although the first PRNP mutations identified, and most since, are PRNP missense, octapeptide repeat insertions, deletion and nonsense mutati …
Fatal Familial Insomnia: Clinical Aspects and Molecular Alterations.
Llorens F, Zarranz JJ, Fischer A, Zerr I, Ferrer I. Llorens F, et al. Curr Neurol Neurosci Rep. 2017 Apr;17(4):30. doi: 10.1007/s11910-017-0743-0. Curr Neurol Neurosci Rep. 2017. PMID: 28324299 Review.
PURPOSE OF REVIEW: Fatal familiar insomnia (FFI) is an autosomal dominant inherited prion disease caused by D178N mutation in the prion protein gene (PRNP D178N) accompanied by the presence of a methionine at the codon 129 polymorphic site on the mutated allele. ...
PURPOSE OF REVIEW: Fatal familiar insomnia (FFI) is an autosomal dominant inherited prion disease caused by D178N mutation in …
Fatal familial insomnia and Agrypnia Excitata: Autonomic dysfunctions and pathophysiological implications.
Baldelli L, Provini F. Baldelli L, et al. Auton Neurosci. 2019 May;218:68-86. doi: 10.1016/j.autneu.2019.02.007. Epub 2019 Feb 26. Auton Neurosci. 2019. PMID: 30890351 Review.
Fatal Familial Insomnia (FFI) is a hereditary prion disease caused by a mutation at codon 178 of the prion-protein gene leading to a D178N substitution in the protein determining severe and selective atrophy of mediodorsal and anteroventral thalamic nuclei. . …
Fatal Familial Insomnia (FFI) is a hereditary prion disease caused by a mutation at codon 178 of the prion-protein gene …
Prionic diseases.
Araújo AQ. Araújo AQ. Arq Neuropsiquiatr. 2013 Sep;71(9B):731-7. doi: 10.1590/0004-282X201301461. Arq Neuropsiquiatr. 2013. PMID: 24141515 Free article. Review.
The prionopathies [Kuru, Creutzfeldt-Jakob disease (CJD) and its variants, Gerstmann-Straussler-Scheinker (GSS) syndrome and fatal familial insomnia (FFI)] result from accumulation of abnormal isoforms of the prion protein in the brains of normal animals on b …
The prionopathies [Kuru, Creutzfeldt-Jakob disease (CJD) and its variants, Gerstmann-Straussler-Scheinker (GSS) syndrome and fatal …
Prion diseases.
Takada LT, Geschwind MD. Takada LT, et al. Semin Neurol. 2013 Sep;33(4):348-56. doi: 10.1055/s-0033-1359314. Epub 2013 Nov 14. Semin Neurol. 2013. PMID: 24234356 Review.
They can be sporadic (Jakob-Creutzfeldt disease [JCD]), genetic (genetic JCD, Gerstmann-Straussler-Scheinker, and familial fatal insomnia), or acquired (kuru, variant JCD, and iatrogenic JCD). ...
They can be sporadic (Jakob-Creutzfeldt disease [JCD]), genetic (genetic JCD, Gerstmann-Straussler-Scheinker, and familial fatal …
168 results