Wikia

Psychology Wiki

Gerstmann-Sträussler-Scheinker syndrome

Talk0
34,142pages on
this wiki

Assessment | Biopsychology | Comparative | Cognitive | Developmental | Language | Individual differences | Personality | Philosophy | Social |
Methods | Statistics | Clinical | Educational | Industrial | Professional items | World psychology |

Clinical: Approaches · Group therapy · Techniques · Types of problem · Areas of specialism · Taxonomies · Therapeutic issues · Modes of delivery · Model translation project · Personal experiences ·


Gerstmann-Sträussler-Scheinker syndrome
ICD-10 A819
ICD-9 046.8
OMIM 137440
DiseasesDB 30729
MedlinePlus [1]
eMedicine /
MeSH {{{MeshNumber}}}

Gerstmann-Sträussler-Scheinker syndrome (GSS) is a very rare, usually familial, fatal neurodegenerative disease that affects patients from 20 to 60 years in age. This extremely rare disease is classified as a transmissible spongiform encephalopathy (TSE).

The exact incidence of GSS is unknown but is estimated to be between 1 to 10 per 100 million. Familial cases are associated with autosomal dominant inheritance.

CausesEdit

It is one of the few diseases which are caused by prions; a class of pathogenic proteins highly resistant to proteases.

A change in codon 102 from proline to leucine has been found in the prion protein gene (PRNP) of most affected individuals. Therefore, it appears this genetic change is usually required for the development of the disease.

SymptomsEdit

Symptoms start with slowly developing dysarthria (difficulty speaking) and cerebellar ataxia (unsteadiness) and then the progressive dementia becomes more evident.

PrognosisEdit

There is no cure or treatment for GSS and patients rarely survive longer than five years.

External linksEdit

  • Gerstmann-Sträussler-Scheinker syndrome, MedicineNet.com
  • UK CJD Surveillance Unit Monitors UK GSS cases and gives a comprehensive list of relevant links.
  • Collins et al (2001), Gerstmann-Sträussler-Scheinker syndrome,fatal familial insomnia, and kuru: a review of these less common human transmissible spongiform encephalopathies. Journal of Clinical Neuroscience 8 (5): 387-397
    PMID 11535002

Template:Prion diseases

This page uses Creative Commons Licensed content from Wikipedia (view authors).

Around Wikia's network

Random Wiki