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

Absence of Evidence for a Causal Link between Bovine Spongiform Encephalopathy Strain Variant L-BSE and Known Forms of Sporadic Creutzfeldt-Jakob Disease in Human PrP Transgenic Mice.
 

Author(s): Emilie Jaumain, Isabelle Quadrio, Laetitia Herzog, Fabienne Reine, Human Rezaei, Olivier Andréoletti, Hubert Laude, Armand Perret-Liaudet, Stéphane Haïk, Vincent Béringue

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Prions are proteinaceous pathogens responsible for subacute spongiform encephalopathies in animals and humans. The prions responsible for bovine spongiform encephalopathy (BSE) are zoonotic agents, causing variant Creutzfeldt-Jakob disease (CJD) in humans. The transfer of prions between ...

Last Updated: 29 Sep 2016

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Guinea Pig Prion Protein Supports Rapid Propagation of Bovine Spongiform Encephalopathy and Variant Creutzfeldt-Jakob Disease Prions.
 

Author(s): Joel C Watts, Kurt Giles, Daniel J Saltzberg, Brittany N Dugger, Smita Patel, Abby Oehler, Sumita Bhardwaj, Andrej Sali, Stanley B Prusiner

Journal:

 

The biochemical and neuropathological properties of bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) prions are faithfully maintained upon transmission to guinea pigs. However, primary and secondary transmissions of BSE and vCJD in guinea pigs result ...

Last Updated: 21 Jul 2016

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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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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 22 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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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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[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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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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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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Enhanced CJD Surveillance in the Older Population
 

Status: Recruiting

Condition Summary: Prion Disease; Dementia

 

Last Updated: 16 May 2017

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