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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Advocacy and Support Organizations

 

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 89 free, full-text research articles on human participants. First 3 results:

Brain-Specific Superoxide Dismutase 2 Deficiency Causes Perinatal Death with Spongiform Encephalopathy in Mice.
 

Author(s): Naotaka Izuo, Hidetoshi Nojiri, Satoshi Uchiyama, Yoshihiro Noda, Satoru Kawakami, Shuji Kojima, Toru Sasaki, Takuji Shirasawa, Takahiko Shimizu

Journal: Oxid Med Cell Longev. 2015 ;2015():238914.

 

Oxidative stress is believed to greatly contribute to the pathogenesis of various diseases, including neurodegeneration. Impairment of mitochondrial energy production and increased mitochondrial oxidative damage are considered early pathological events that lead to neurodegeneration. ...

Last Updated: 24 Aug 2015

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Further characterisation of transmissible spongiform encephalopathy phenotypes after inoculation of cattle with two temporally separated sources of sheep scrapie from Great Britain.
 

Author(s): Timm Konold, Romolo Nonno, John Spiropoulos, Melanie J Chaplin, Michael J Stack, Steve A C Hawkins, Saira Cawthraw, John W Wilesmith, Gerald A H Wells, Umberto Agrimi, Michele A Di Bari, Olivier Andréoletti, Juan C Espinosa, Patricia Aguilar-Calvo, Juan M Torres

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The infectious agent responsible for the bovine spongiform encephalopathy (BSE) epidemic in Great Britain is a transmissible spongiform encephalopathy (TSE) strain with uniform properties but the origin of this strain remains unknown. Based on the hypothesis that classical BSE may ...

Last Updated: 24 Jul 2015

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Insights into Chronic Wasting Disease and Bovine Spongiform Encephalopathy Species Barriers by Use of Real-Time Conversion.
 

Author(s): Kristen A Davenport, Davin M Henderson, Jifeng Bian, Glenn C Telling, Candace K Mathiason, Edward A Hoover

Journal: J. Virol.. 2015 Sep;89(18):9524-31.

 

The propensity for transspecies prion transmission is related to the structural characteristics of the enciphering and new host PrP, although the exact mechanism remains incompletely understood. The effects of variability in prion protein on cross-species prion transmission have been ...

Last Updated: 21 Aug 2015

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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 27 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, Jürgen 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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Reflections on a half-century in the field of transmissible spongiform encephalopathy.
 

Author(s): Paul Brown

Journal: Folia Neuropathol. 2009 ;47(2):95-103.

 

The subject of transmissible spongiform encephalopathy may properly be said to have begun with the experimental transmission of scrapie by Cuillé and Chelle in 1936, although Creutzfeldt and Jakob had described the disease that bears their names in 1920-21. Thirty more years passed ...

Last Updated: 20 Jul 2009

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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Therapeutic Antibodies Against Prion Diseases From PRNP Mutation Carriers
 

Status: Recruiting

Condition Summary: Prion Diseases

 

Last Updated: 14 Jul 2016

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Enhanced CJD Surveillance in the Older Population
 

Status: Recruiting

Condition Summary: Prion Disease; Dementia

 

Last Updated: 26 Jan 2016

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The Role of the Coagulation Pathway at the Synapse in Prion Diseases
 

Status: Not yet recruiting

Condition Summary: Creutzfeldt-Jakob Syndrome

 

Last Updated: 23 Jun 2015

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