Genetic and Rare Diseases Information Center (GARD)


Other names people use for this condition
  • Gerstmann-Straussler-Scheinker disease
  • Gerstmann Straussler Scheinker syndrome
  • Cerebellar ataxia, progressive dementia, and amyloid deposits in the central nervous system
  • Encephalopathy subacute spongiform Gerstmann-Straussler type
  • Amyloidosis cerebral with spongiform encephalopathy
  • GSSD
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Home > Genetic & Rare Diseases Information Center (GARD) > Spongiform encephalopathy



Spongiform encephalopathy
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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.[1]

References
  1. NINDS Gerstmann-Straussler-Scheinker Disease Information Page. National Institute of Neurological Disorders and Stroke (NINDS). February 13, 2007 Available at: http://www.ninds.nih.gov/disorders/gss/gss.htm. Accessed March 21, 2008.

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For more information about Spongiform encephalopathy click on the boxes below:
More Detailed Information NLM Gateway Services Support Groups Clinical Trials & Research
 General | Management Guidelines | Selected Full-Text Journal Articles
More Detailed Information (Found 12 resources)
Links where you can find more general information, comprehensive resources, selected full text journal articles, and news updates

  • General

    • eMedicine provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free
    • Genetics Home Reference (GHR) contains a condition summary on Spongiform encephalopathy. Click on the link to go to GHR and review this summary.
    • The Merck Manuals Online Medical Library provides information on this condition. Click on the link to view the information.
    • MeSH® (Medical Subject Headings) is a terminology tool used by the National Library of Medicine. Click on the link to view information on this topic.
    • The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.
    • The Online Mendelian Inheritance in Man (OMIM) database contains genetics resources that discuss Spongiform encephalopathy. Click on the link to go to OMIM and review these resources.
    • Orphanet is a database dedicated to information on rare diseases and orphan drugs.  Access to this database is free of charge.  Click on the link to read information on this topic.
    • PubMed is a searchable database of medical literature and lists journal articles that discuss Spongiform encephalopathy. Click on the link to view a sample search on this topic.
    • The Social Security Administration (SSA) created the Compassionate Allowance Initiative to speed up the processing of disability claims for applicants with certain conditions. Click on the link to view the Compassionate Allowance information for this condition. More information about applying for Social Security disability benefits is available online.
      Link: https://secure.ssa.gov/apps10/poms.nsf/lnx/0423022145
    • The National Institute of Neurological Disorders and Stroke (NINDS), part of the National Institutes of Health (NIH), has an information page on Gerstmann-Straussler-Scheinker disease.  To read more about Gerstmann-Straussler-Scheinker disease, visit the following link: http://www.ninds.nih.gov/disorders/gss/gss.htm


      PO Box 5801
      Bethesda, MD 20824
      Toll free:  800-352-9424
      Web site:   http://www.ninds.nih.gov/
  • Management Guidelines

    • GeneReviews provides current, expert-authored, peer-reviewed, full-text articles describing the application of genetic testing to the diagnosis, management, and genetic counseling of patients with specific inherited conditions. Click on the link to view the article on this topic.
  • Selected Full-Text Journal Articles

 
NLM Gateway (Found 1 resource)
A tool to search across multiple resources offered on the National Library of Medicine's Website

    • NLM Gateway allows users to search many resources offered on the National Library of Medicine's Web site at once to quickly find more information about this condition. Some of the resources may be a duplicate of the resources listed on this page. To search NLM Gateway, click on the link; the condition name will already be in the search box, so you can just click the “Search” button.
 Testing | Genetic Services
Services (Found 2 resources)
Links to Web sites that offer services, such as tools to locate specialists, specialty clinics, genetic services, and genetic testing laboratories

  • Testing

    • GeneTests lists laboratories offering clinical genetic testing for this condition. Clinical genetic tests are ordered to help diagnose a person or family and to aid in decisions regarding medical care or reproductive issues. Talk to your health care provider or a genetic professional to learn more about your testing options.
  • Genetic Services

    • We recommend that you discuss this information and your concerns with a genetics professional. The following online resources can help you find a genetics professional in your community:

       * GeneClinics - A searchable directory of US and international genetics and prenatal diagnosis clinics. To locate genetics clinics in the United States, go to the following link and click on 'Clinic Directory' to find a genetic service close to you. 

        * ResourceLink - A database of genetics counseling services, searchable by location, name, institution, type of practice, or specialty. Hosted by the National Society of Genetic Counselors.
 Umbrella Organizations | Live Chat/E-mail Lists | Parent Matching Organizations
Support Groups (Found 5 resources)
Groups providing a wide range of services, supportive resources, and information

  • Umbrella Organizations

  • Live Chat/E-mail Lists

    • The National Organization of Rare Disorders (NORD) has partnered with Inspire.com to launch an online community for people with rare diseases called The NORD Rare Disease Community. This community connects medical patients, family members, caregivers, and professionals. Click on The NORD Rare Disease Community to learn more.
    • RareShare is an online social hub dedicated to patients, families and healthcare professionals affected by rare medical disorders. Click on RareShare to learn more.
  • Parent Matching Organizations

 
Clinical Trials & Research (Found 1 resource)
Resources where you may find research studies and clinical trials

    • ClinicalTrials.gov lists trials that are studying or have studied Spongiform encephalopathy. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

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