Spongiform encephalopathy

Common Name(s)

Spongiform encephalopathy, Gerstmann Straussler Scheinker syndrome, Gerstmann-Straussler-Scheinker disease, Amyloidosis cerebral with spongiform encephalopathy

Gerstmann-Straussler-Scheinker disease is a neurodegenerative brain disorder which causes ataxia and dementia. It is almost always inherited and is found in only a few families around the world. Onset of the disease usually occurs between the ages of 35 and 55. Gerstmann-Straussler-Scheinker disease belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs) or prion diseases. Other TSEs include Creutzfeldt-Jakob disease, kuru, and fatal familial insomnia.
 

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Condition Specific Organizations

Following organizations serve the condition "Spongiform encephalopathy" for support, advocacy or research.

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Spongiform encephalopathy" returned 85 free, full-text research articles on human participants. First 3 results:

Increased susceptibility of transgenic mice expressing human PrP to experimental sheep bovine spongiform encephalopathy is not due to increased agent titre in sheep brain tissue.
 

Author(s): Chris Plinston, Patricia Hart, Nora Hunter, Jean C Manson, Rona M Barron

Journal: J. Gen. Virol.. 2014 Aug;95(Pt 8):1855-9.

 

Bovine spongiform encephalopathy (BSE) in cattle and variant Creutzfeldt-Jakob disease in humans have previously been shown to be caused by the same strain of transmissible spongiform encephalopathy agent. It is hypothesized that the agent spread to humans following consumption of ...

Last Updated: 19 Jul 2014

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Prevalent abnormal prion protein in human appendixes after bovine spongiform encephalopathy epizootic: large scale survey.
 

Author(s): O Noel Gill, Yvonne Spencer, Angela Richard-Loendt, Carole Kelly, Reza Dabaghian, Lynnette Boyes, Jacqueline Linehan, Marion Simmons, Paul Webb, Peter Bellerby, Nick Andrews, David A Hilton, James W Ironside, Jon Beck, Mark Poulter, Simon Mead, Sebastian Brandner

Journal:

 

To carry out a further survey of archived appendix samples to understand better the differences between existing estimates of the prevalence of subclinical infection with prions after the bovine spongiform encephalopathy epizootic and to see whether a broader birth cohort was affected, ...

Last Updated: 16 Oct 2013

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Dissociation of prion protein amyloid seeding from transmission of a spongiform encephalopathy.
 

Author(s): Pedro Piccardo, Declan King, Glenn Telling, Jean C Manson, Rona M Barron

Journal: J. Virol.. 2013 Nov;87(22):12349-56.

 

Misfolding and aggregation of proteins are common pathogenic mechanisms of a group of diseases called proteinopathies. The formation and spread of proteinaceous lesions within and between individuals were first described in prion diseases and proposed as the basis of their infectious ...

Last Updated: 18 Oct 2013

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Spongiform encephalopathy" returned 20 free, full-text review articles on human participants. First 3 results:

Laboratory activities involving transmissible spongiform encephalopathy causing agents: risk assessment and biosafety recommendations in Belgium.
 

Author(s): Amaya Leunda, Bernadette Van Vaerenbergh, Aline Baldo, Stefan Roels, Philippe Herman

Journal: Prion. ;7(5):420-33.

 

Since the appearance in 1986 of epidemic of bovine spongiform encephalopathy (BSE), a new form of neurological disease in cattle which also affected human beings, many diagnostic and research activities have been performed to develop detection and therapeutic tools. A lot of progress ...

Last Updated: 13 Jan 2014

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Experimental interspecies transmission studies of the transmissible spongiform encephalopathies to cattle: comparison to bovine spongiform encephalopathy in cattle.
 

Author(s): Amir N Hamir, Marcus E Kehrli, Robert A Kunkle, Justin J Greenlee, Eric M Nicholson, J├╝rgen A Richt, Janice M Miller, Randall C Cutlip

Journal: J. Vet. Diagn. Invest.. 2011 May;23(3):407-20.

 

Prion diseases or transmissible spongiform encephalopathies (TSEs) of animals include scrapie of sheep and goats; transmissible mink encephalopathy (TME); chronic wasting disease (CWD) of deer, elk and moose; and bovine spongiform encephalopathy (BSE) of cattle. The emergence of BSE ...

Last Updated: 12 Sep 2011

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[Prions: where do we stand 20 years after the appearance of bovine spongiform encephalopathy?].
 

Author(s): Carole Crozet, Sylvain Lehmann

Journal: Med Sci (Paris). 2007 Dec;23(12):1148-57.

 

Bovine spongiform encephalopathy (BSE) is a transmissible spongiform encephalopathy (TSE) identified twenty years ago in the British cattle herds. Creutzfeldt-Jakob disease (CJD) is a TSE that occurs in humans. In 1996, scientists found a possible link between BSE and a new variant ...

Last Updated: 24 Dec 2007

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Last Updated: 5 Apr 2014

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Notification of Donors With Positive Microbiology Markers
 

Status: Not yet recruiting

Condition Summary: Human Immunodeficiency Virus; Hepatitis B; Hepatitis C; Human T-lymphotropic Virus I & II; Creutzfeldt-Jakob Syndrome; HIV Infections

 

Last Updated: 12 Feb 2010

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