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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Gerstmann-Straussler-Scheinker disease


Other Names for this Disease

  • Amyloidosis cerebral with spongiform encephalopathy
  • Cerebellar ataxia, progressive dementia, and amyloid deposits in the central nervous system
  • Encephalopathy subacute spongiform Gerstmann-Straussler type
  • Gerstmann Straussler Scheinker syndrome
  • GSSD
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

What is Gerstmann-Straussler-Scheinker disease?

What are the symptoms of Gerstmann-Straussler-Scheinker disease?

How is Gerstmann-Straussler-Scheinker disease inherited?

How might Gerstmann-Straussler-Scheinker disease be treated?

What is Gerstmann-Straussler-Scheinker disease?

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]
Last updated: 7/22/2013

What are the symptoms of Gerstmann-Straussler-Scheinker disease?

In the early stages of Gerstmann-Straussler-Scheinker disease, patients may experience varying levels of ataxia (lack of muscle coordination), including clumsiness, unsteadiness, and difficulty walking. As the disease progresses, the ataxia becomes more pronounced and most patients develop dementia. Other symptoms may include dysarthria (slurring of speech), nystagmus (involuntary movements of the eyes), spasticity (rigid muscle tone), and visual disturbances, sometimes leading to blindness. Deafness also can occur. In some families, parkinsonian features are present.[1]
Last updated: 7/22/2013

How is Gerstmann-Straussler-Scheinker disease inherited?

Gerstmann-Straussler-Scheinker disease is inherited in an autosomal dominant fashion, which means one copy of the altered gene in each cell is sufficient to cause the disorder. In most cases, an affected person inherits the altered gene from one affected parent. In some people, Gerstmann-Straussler-Scheinker disease is caused by a new mutation. Although these individuals most likely do not have an affected parent, they can pass the genetic change to their children.[2]
Last updated: 7/22/2013

How might Gerstmann-Straussler-Scheinker disease be treated?

There is no cure for Gerstmann-Straussler-Scheinker disease, nor are there any known treatments to slow its progression. Current therapies are aimed at alleviating symptoms and making the patient as comfortable as possible.[1]
Last updated: 7/22/2013

References
  1. NINDS Gerstmann-Straussler-Scheinker Disease Information Page. National Institute of Neurological Disorders and Stroke (NINDS). February 13, 2007; http://www.ninds.nih.gov/disorders/gss/gss.htm. Accessed 3/21/2008.
  2. Prion disease. Genetics Home Reference (GHR). May 2007; http://ghr.nlm.nih.gov/condition=priondisease. Accessed 3/21/2008.


Other Names for this Disease
  • Amyloidosis cerebral with spongiform encephalopathy
  • Cerebellar ataxia, progressive dementia, and amyloid deposits in the central nervous system
  • Encephalopathy subacute spongiform Gerstmann-Straussler type
  • Gerstmann Straussler Scheinker syndrome
  • GSSD
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.