here we go again...
A new prion disease: relationship with central and peripheral
amyloidoses
Simon Mead1, & Mary M. Reilly2,
Affiliations
Contributions
Corresponding author
Journal name: Nature Reviews Neurology
Year published: (2015)
DOI: doi:10.1038/nrneurol.2014.263
Published online 27 January 2015
Abstract
Prion diseases are typically recognized as rapidly progressive dementing
illnesses that also feature myoclonus and cerebellar ataxia. Several families
have now been described with a late-onset hereditary sensory and autonomic
neuropathy caused by truncation of prion protein (PrP), and associated with
systemic amyloidosis, which was a profoundly unexpected phenotype. The chronic
symptoms of this disorder, termed PrP systemic amyloidosis, can be very
disabling, and are comparable to familial amyloid polyneuropathy (FAP) caused by
transthyretin mutations. Patients require symptomatic therapies directed towards
control of nausea, diarrhoea, incontinence, neuropathic pain and postural
hypotension. Although the potential transmissibility of this new prion disease
is probably extremely low, we advocate PrP gene analysis before biopsy in the
investigation of peripheral and autonomic neuropathies, or for patients with
unexplained diarrhoea and neuropathy. Prion diseases and the FAPs both display
prominent effects of mutation type on clinical presentation and patterns of
pathology—a fascinating but unexplained observation. Several neurodegenerative
diseases associated with central protein misfolding, such as Huntington and
Parkinson diseases, also have under-recognized peripheral components. Most of
the familial amyloidoses can be explained by known gene mutations, but amino
acid variants in proteins involved in other central neurodegenerative diseases
might direct the initial pathology to the periphery.
Self-Propagative Replication of Ab Oligomers Suggests Potential
Transmissibility in Alzheimer Disease
Received July 24, 2014; Accepted September 16, 2014; Published November 3,
2014
Singeltary comment ;
Monday, November 17, 2014
Prion-like transmission and spreading of tau pathology
Tuesday, July 1, 2014
Distinct synthetic Aβ prion strains producing different amyloid deposits in
bigenic mice
Tuesday, November 26, 2013
Transmission of multiple system atrophy prions to transgenic mice
Tuesday, December 17, 2013
Alzheimer's Disease U.K. diagnosed by region in each of the last five years
[179852]
Wednesday, June 19, 2013
Spreading of tau pathology in Alzheimer's disease by cell-to-cell
transmission
Saturday, May 25, 2013
Brain homogenates from human tauopathies induce tau inclusions in mouse
brain
Saturday, May 25, 2013
Brain homogenates from human tauopathies induce tau inclusions in mouse
brain
Sunday, February 10, 2013
Parkinson's Disease and Alpha Synuclein: Is Parkinson's Disease a
Prion-Like Disorder?
Wednesday, January 5, 2011
ENLARGING SPECTRUM OF PRION-LIKE DISEASES Prusiner Colby et al 2011 Prions
David W. Colby1,* and Stanley B. Prusiner1,2
Friday, September 3, 2010
Alzheimer's, Autism, Amyotrophic Lateral Sclerosis, Parkinson's, Prionoids,
Prionpathy, Prionopathy, TSE
Tuesday, November 04, 2014
Towards an Age-Dependent Transmission Model of Acquired and Sporadic
Creutzfeldt-Jakob Disease
Thursday, January 22, 2015
Transmission properties of atypical Creutzfeldt-Jakob disease: a clue to
disease etiology?
Friday, January 10, 2014
vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type
prion disease, what it ???
Monday, November 3, 2014
USA CJD TSE PRION UNIT, TEXAS, SURVEILLANCE UPDATE NOVEMBER 2014
National Prion Disease Pathology Surveillance Center Cases Examined1
(October 7, 2014)
***6 Includes 11 cases in which the diagnosis is pending, and 19
inconclusive cases;
***7 Includes 12 (11 from 2014) cases with type determination pending in
which the diagnosis of vCJD has been excluded.
***The sporadic cases include 2660 cases of sporadic Creutzfeldt-Jakob
disease (sCJD),
***50 cases of Variably Protease-Sensitive Prionopathy (VPSPr)
***and 21 cases of sporadic Fatal Insomnia (sFI).
Sunday, December 14, 2014
ALERT new variant Creutzfeldt Jakob Disease nvCJD or vCJD, sporadic CJD
strains, TSE prion aka Mad Cow Disease United States of America Update December
14, 2014 Report
Saturday, December 13, 2014
*** Terry S. Singeltary Sr. Publications TSE prion disease Peer Review
***
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14,
2001 JAMA
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TSS
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