 Gerstmann-Straussler-Scheinker Disease
"Gerstmann-Straussler-Scheinker Disease" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus,
MeSH (Medical Subject Headings). Descriptors are arranged in a hierarchical structure,
which enables searching at various levels of specificity.
An autosomal dominant familial prion disease with a wide spectrum of clinical presentations including ATAXIA, spastic paraparesis, extrapyramidal signs, and DEMENTIA. Clinical onset is in the third to sixth decade of life and the mean duration of illness prior to death is five years. Several kindreds with variable clinical and pathologic features have been described. Pathologic features include cerebral prion protein amyloidosis, and spongiform or neurofibrillary degeneration. (From Brain Pathol 1998 Jul;8(3):499-513; Brain Pathol 1995 Jan;5(1):61-75)
| Descriptor ID |
D016098
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| MeSH Number(s) |
C10.228.228.800.350 C10.574.500.425 C10.574.843.400 C16.320.400.350
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| Concept/Terms |
Gerstmann-Straussler-Scheinker Disease- Gerstmann-Straussler-Scheinker Disease
- Gerstmann Straussler Scheinker Disease
- Inherited Spongiform Encephalopathy, Gerstmann-Straussler
- Inherited Spongiform Encephalopathy, Gerstmann Straussler
- Gerstmann-Straussler Syndrome
- Gerstmann Straussler Syndrome
- Gerstmann-Straussler-Scheinker Syndrome
- Gerstmann Straussler Scheinker Syndrome
- Encephalopathy, Subacute Spongiform, Gerstmann-Straussler Type
- Gerstmann-Straussler Inherited Spongiform Encephalopathy
- Gerstmann Straussler Inherited Spongiform Encephalopathy
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Below are MeSH descriptors whose meaning is more general than "Gerstmann-Straussler-Scheinker Disease".
Below are MeSH descriptors whose meaning is more specific than "Gerstmann-Straussler-Scheinker Disease".
This graph shows the total number of publications written about "Gerstmann-Straussler-Scheinker Disease" by people in this website by year, and whether "Gerstmann-Straussler-Scheinker Disease" was a major or minor topic of these publications.
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| Year | Major Topic | Minor Topic | Total |
|---|
| 1995 | 1 | 1 | 2 | | 1998 | 1 | 1 | 2 | | 2000 | 0 | 2 | 2 | | 2009 | 1 | 0 | 1 | | 2010 | 0 | 1 | 1 | | 2012 | 1 | 0 | 1 | | 2018 | 1 | 0 | 1 | | 2019 | 1 | 1 | 2 |
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Below are the most recent publications written about "Gerstmann-Straussler-Scheinker Disease" by people in Profiles.
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Qin K, Zhao L, Gregory C, Solanki A, Mastrianni JA. "Dual Disease" TgAD/GSS mice exhibit enhanced Alzheimer's disease pathology and reveal PrPC-dependent secretion of Aß. Sci Rep. 2019 06 12; 9(1):8524.
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Qin K, Zhao L, Solanki A, Busch C, Mastrianni J. Anle138b prevents PrP plaque accumulation in Tg(PrP-A116V) mice but does not mitigate clinical disease. J Gen Virol. 2019 06; 100(6):1027-1037.
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Gu H, Kirchhein Y, Zhu T, Zhao G, Peng H, Du E, Liu J, Mastrianni JA, Farlow MR, Dodel R, Du Y. IVIG Delays Onset in a Mouse Model of Gerstmann-Sträussler-Scheinker Disease. Mol Neurobiol. 2019 Apr; 56(4):2353-2361.
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Cortes CJ, Qin K, Cook J, Solanki A, Mastrianni JA. Rapamycin delays disease onset and prevents PrP plaque deposition in a mouse model of Gerstmann-Sträussler-Scheinker disease. J Neurosci. 2012 Sep 05; 32(36):12396-405.
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Brown K, Mastrianni JA. The prion diseases. J Geriatr Psychiatry Neurol. 2010 Dec; 23(4):277-98.
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Yang W, Cook J, Rassbach B, Lemus A, DeArmond SJ, Mastrianni JA. A New Transgenic Mouse Model of Gerstmann-Straussler-Scheinker Syndrome Caused by the A117V Mutation of PRNP. J Neurosci. 2009 Aug 12; 29(32):10072-80.
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Mastrianni JA, Roos RP. The prion diseases. Semin Neurol. 2000; 20(3):337-52.
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Worrall BB, Rowland LP, Chin SS, Mastrianni JA. Amyotrophy in prion diseases. Arch Neurol. 2000 Jan; 57(1):33-8.
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Hegde RS, Mastrianni JA, Scott MR, DeFea KA, Tremblay P, Torchia M, DeArmond SJ, Prusiner SB, Lingappa VR. A transmembrane form of the prion protein in neurodegenerative disease. Science. 1998 Feb 06; 279(5352):827-34.
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Mastrianni JA. The prion diseases: Creutzfeldt-Jakob, Gerstmann-Sträussler-Scheinker, and related disorders. J Geriatr Psychiatry Neurol. 1998; 11(2):78-97.
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