Gerstmann-Straussler-Scheinker disease
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At the time the article was created Henry Knipe had no recorded disclosures.
View Henry Knipe's current disclosuresAt the time the article was last revised Frank Gaillard had no recorded disclosures.
View Frank Gaillard's current disclosures- Gerstmann-Straussler-Scheinker syndrome
Gerstmann-Straussler-Scheinker disease is a very rare type of 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.
History and etymology
It is named after the Austrian-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.
References
- 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. doi:10.1111/j.1365-2990.2011.01174.x
- 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. doi:10.3988/jcn.2010.6.1.46
- 3. Witold K Surewicz P. Molecular Genetics of Gerstmann-Straussler-Scheinker Disease and Creutzfeld-Jakob Disease. Hereditary Genetics. 2013;02(02). doi:10.4172/2161-1041.1000117
- 4. Triarhou L. Ernst Sträussler (1872-959). J Neurol. 2007;254(10):1466-7. doi:10.1007/s00415-007-0681-x
- 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. doi:10.1017/cjn.2015.359
- 6. Triarhou L. Josef Gerstmann (1887–1969). J Neurol. 2007;255(4):614-5. doi:10.1007/s00415-008-0851-5
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