Gerstmann-Straussler-Scheinker disease
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Gerstmann-Straussler-Scheinker disease is a very rare type of human transmissible spongiform encephalopathy. It manifests with dementia and/or ataxia and is due to a mutation in the prion protein (PRNP)) gene, which is inherited in an autosomal dominant pattern.
-<p><strong>Gerstmann-Straussler-Scheinker disease</strong> is a very rare type of human transmissible <a title="spongiform encephalopathy" href="/articles/spongiform-encephalopathy">spongiform encephalopathy</a>. It manifests with dementia and/or ataxia and is due to a mutation in the prion protein (<em>PRNP</em>) gene, which is inherited in an autosomal dominant pattern. </p>- +<p><strong>Gerstmann-Straussler-Scheinker disease</strong> is a very rare type of human transmissible <a href="/articles/spongiform-encephalopathy">spongiform encephalopathy</a>. It manifests with dementia and/or ataxia and is due to a mutation in the prion protein (<em>PRNP)</em> gene, which is inherited in an autosomal dominant pattern. </p>
References changed:
- 2. Park M, Jo H, Cheon S, Choi S, Kim Y, Kim J. A Case of Gerstmann-Sträussler-Scheinker Disease. J Clin Neurol. 2010;6(1):46. <a href="https://doi.org/10.3988/jcn.2010.6.1.46">doi:10.3988/jcn.2010.6.1.46</a>
- 3. Witold K Surewicz P. Molecular Genetics of Gerstmann-Straussler-Scheinker Disease and Creutzfeld-Jakob Disease. Hereditary Genetics. 2013;02(02). <a href="https://doi.org/10.4172/2161-1041.1000117">doi:10.4172/2161-1041.1000117</a>
- 1. Vital A, Laplanche J, Bastard J, Xiao X, Zou W, Vital C. A Case of Gerstmann-Sträussler-Scheinker Disease with a Novel Six Octapeptide Repeat Insertion. Neuropathology and Applied Neurobiology. 2011;37(5):554-9. <a href="https://doi.org/10.1111/j.1365-2990.2011.01174.x">doi:10.1111/j.1365-2990.2011.01174.x</a>
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- refs
- cases
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- Syndromes
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- Central Nervous System