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 gene, which is inherited in an autosomal dominant pattern.
History and etymology
It is partially named after the AmericanAustrian-American neuropsychiatrist Josef Gerstmann (1887-1969)6, also of Gerstmann syndrome fame, Austrian neuropathologist Ernst Sträussler (1872-1959) 4, and Austrian-American neuropathologist Ilya Mark Scheinker (1902-1954) 5.
-<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><h4>History and etymology</h4><p>It is partially named after the American neuropsychiatrist <strong>Josef Gerstmann</strong> (1887-1969), also of <a title="Gerstmann syndrome" href="/articles/gerstmann-syndrome">Gerstmann syndrome</a> fame.</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><h4>History and etymology</h4><p>It is named after the Austrian-American neuropsychiatrist <strong>Josef Gerstmann</strong> (1887-1969) <sup>6</sup>, also of <a href="/articles/gerstmann-syndrome">Gerstmann syndrome</a> fame, Austrian neuropathologist <strong>Ernst Sträussler</strong> (1872-1959) <sup>4</sup>, and Austrian-American neuropathologist <strong>Ilya Mark Scheinker</strong> (1902-1954) <sup>5</sup>.</p><p> </p>
References changed:
- 4. Triarhou L. Ernst Sträussler (1872-959). J Neurol. 2007;254(10):1466-7. <a href="https://doi.org/10.1007/s00415-007-0681-x">doi:10.1007/s00415-007-0681-x</a>
- 5. Zeidman L, Ziller M, Shevell M. Ilya Mark Scheinker: Controversial Neuroscientist and Refugee From National Socialist Europe. Can J Neurol Sci. 2016;43(2):334-44. <a href="https://doi.org/10.1017/cjn.2015.359">doi:10.1017/cjn.2015.359</a>
- 6. Triarhou L. Josef Gerstmann (1887–1969). J Neurol. 2007;255(4):614-5. <a href="https://doi.org/10.1007/s00415-008-0851-5">doi:10.1007/s00415-008-0851-5</a>
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