Gerstmann-Straussler-Scheinker syndrome - Revision history

Nnjm2: Cleaned up the text.

2017-11-18T08:55:53Z

Cleaned up the text.

← Older revision		Revision as of 08:55, 18 November 2017
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	Gerstmann-Straussler-Scheinker (GSS) syndrome is a neurodegenerative ~~disorder~~<ref>1. National Institute of Neurological Disorders and Stroke (NINDS), Gerstmann-Straussler-Scheinker Disease Information Page, (2007),[Online], Available at: http://www.ninds.nih.gov/disorders/gss/gss.htm. Accessed 3/21/2008, (Last Accessed: 26/11/14)</ref> affecting the [[Brain\|brain]]. This leads to conditions such as ataxia and dementia, which includes common symptoms of memory loss and the loss of balance and coordination, respectively.~~ ~~The disease itself is~~ ~~almost always inherited and is very rare; only a few known cases are reported around the world, running down through families<ref>De Michele G, Pocchiari M, Petraroli R, et al., (August 2003), "Variable phenotype in a P102L Gerstmann–Sträussler–Scheinker Italian family", Can J Neurol Sci 30 (3): 233–6. PMID 12945948</ref> due to inheritance. ~~Onset~~ of the disease usually occurs between the ages of 35 and 55. GSS syndrome belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs) or [[Prion\|prion]] diseases<ref>K Hsiao, C Cass, GD Schellenberg, A prion protein variant in a family with the telencephalic form of Gerstmann–Sträussler–Scheinker syndrome, Neurology, 41 (1991), pp. 681–684</ref>.~~<br>~~		Gerstmann-Straussler-Scheinker (GSS) syndrome is a [[neurodegenerative disorde\|neurodegenerative disorde]]r<ref>1. National Institute of Neurological Disorders and Stroke (NINDS), Gerstmann-Straussler-Scheinker Disease Information Page, (2007),[Online], Available at: http://www.ninds.nih.gov/disorders/gss/gss.htm. Accessed 3/21/2008, (Last Accessed: 26/11/14)</ref> affecting the [[Brain\|brain]]. This leads to conditions such as ataxia and [[dementia\|dementia]], which includes common symptoms of memory loss and the loss of balance and coordination, respectively. The disease itself is almost always inherited and is very rare; only a few known cases are reported around the world, running down through families<ref>De Michele G, Pocchiari M, Petraroli R, et al., (August 2003), "Variable phenotype in a P102L Gerstmann–Sträussler–Scheinker Italian family", Can J Neurol Sci 30 (3): 233–6. PMID 12945948</ref> due to inheritance. The onset of the disease usually occurs between the ages of 35 and 55. GSS syndrome belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs) or [[Prion\|prion]] diseases<ref>K Hsiao, C Cass, GD Schellenberg, A prion protein variant in a family with the telencephalic form of Gerstmann–Sträussler–Scheinker syndrome, Neurology, 41 (1991), pp. 681–684</ref>.

	=== Causes ===		=== Causes ===

	GSS is one of few diseases that are caused by the transformation of~~ ~~prion proteins; often due to small changes in its [[Amino acid\|amino acid]] sequence at~~ ~~a particular [[Codon\|codon]].~~  ~~A change in the~~ ~~amino acid sequence from [[Proline\|Proline]]~~ ~~(P)~~ ~~to [[Leucine\|leucine]]~~ ~~(L)~~ ~~on [[Codon\|codon]] 102 found in [[Chromosome 20\|chromosome 20]]<ref>L Goldfarb, P Brown, A Vrbovská, An insert mutation in the chromosome 20 amyloid precursor gene in a Gerstmann–Sträussler–Scheinker family, J Neurol Sci, 111 (1992), pp. 189–194</ref>,~~ ~~is~~ ~~observed in the prion protein gene (PRNP) of most affected individuals. From current knowledge, this genetic change is usually required for the development of GSS.~~ ~~Genetic~~ ~~testing allows for the [[Diagnosis\|diagnosis]] of this underlying genetic mutation. This involves a [[Blood\|blood]] and [[DNA\|DNA]] sample from a potential patient and examining it~~ ~~in order to attempt to detect this mutated gene at certain codons. If the genetic mutation is present, the patient will eventually be afflicted by GSS, and, due to the genetic or inheritance-based nature of the disease, the offspring of the patient ~~have~~ a much higher risk of inheriting the Proline to Leucine mutation, thus contracting GSS.~~<br>~~		GSS is one of few diseases that are caused by the transformation of prion proteins; often due to small changes in its [[Amino acid\|amino acid]] sequence at a particular [[Codon\|codon]]. A change in the amino acid sequence from [[Proline\|Proline]] (P) to [[Leucine\|leucine]] (L) on [[Codon\|codon]] 102 found in [[Chromosome 20\|chromosome 20]]<ref>L Goldfarb, P Brown, A Vrbovská, An insert mutation in the chromosome 20 amyloid precursor gene in a Gerstmann–Sträussler–Scheinker family, J Neurol Sci, 111 (1992), pp. 189–194</ref>, is observed in the prion protein gene (PRNP) of most affected individuals. From current knowledge, this genetic change is usually required for the development of GSS. Genetic testing allows for the [[Diagnosis\|diagnosis]] of this underlying genetic mutation. This involves a [[Blood\|blood]] and [[DNA\|DNA]] sample from a potential patient and examining it in order to attempt to detect this mutated gene at certain codons. If the genetic mutation is present, the patient will eventually be afflicted by GSS, and, due to the genetic or inheritance-based nature of the disease, the offspring of the patient has a much higher risk of inheriting the Proline to Leucine mutation, thus contracting GSS.

	=== Treatment ===		=== Treatment ===

	There is no known cure available GSS, and there are no~~ ~~known treatments to slow the progression of the disease. However, therapies and medication are aimed at treating or slowing down~~ ~~and alleviating the symptoms, which will hopefully~~ ~~lead to an increased quality of life for the patient.~~<br>~~		There is no known cure available GSS, and there are no known treatments to slow the progression of the disease. However, therapies and medication are aimed at treating or slowing down and alleviating the symptoms, which will hopefully lead to an increased quality of life for the patient.

	=== References ===		=== References ===

	<references /~~><br~~>		<references />

170083115: Cleaned up punctuation in reference (before full stop).

2017-11-17T11:57:03Z

Cleaned up punctuation in reference (before full stop).

← Older revision		Revision as of 11:57, 17 November 2017
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	Gerstmann-Straussler-Scheinker (GSS) syndrome is a neurodegenerative disorder<ref>1. National Institute of Neurological Disorders and Stroke (NINDS), Gerstmann-Straussler-Scheinker Disease Information Page, (2007),[Online], Available at: http://www.ninds.nih.gov/disorders/gss/gss.htm. Accessed 3/21/2008, (Last Accessed: 26/11/14)</ref> affecting the [[Brain\|brain]]. This leads to conditions such as ataxia and dementia, which includes common symptoms of memory loss and the loss of balance and coordination, respectively. The disease itself is almost always inherited and is very rare; only a few known cases are reported around the world, running down through families<ref>De Michele G, Pocchiari M, Petraroli R, et al., (August 2003), "Variable phenotype in a P102L Gerstmann–Sträussler–Scheinker Italian family", Can J Neurol Sci 30 (3): 233–6. PMID 12945948</ref> due to inheritance. Onset of the disease usually occurs between the ages of 35 and 55. GSS syndrome belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs) or [[Prion\|prion]] diseases.<ref>K Hsiao, C Cass, GD Schellenberg, A prion protein variant in a family with the telencephalic form of Gerstmann–Sträussler–Scheinker syndrome, Neurology, 41 (1991), pp. 681–684</ref><br>		Gerstmann-Straussler-Scheinker (GSS) syndrome is a neurodegenerative disorder<ref>1. National Institute of Neurological Disorders and Stroke (NINDS), Gerstmann-Straussler-Scheinker Disease Information Page, (2007),[Online], Available at: http://www.ninds.nih.gov/disorders/gss/gss.htm. Accessed 3/21/2008, (Last Accessed: 26/11/14)</ref> affecting the [[Brain\|brain]]. This leads to conditions such as ataxia and dementia, which includes common symptoms of memory loss and the loss of balance and coordination, respectively. The disease itself is almost always inherited and is very rare; only a few known cases are reported around the world, running down through families<ref>De Michele G, Pocchiari M, Petraroli R, et al., (August 2003), "Variable phenotype in a P102L Gerstmann–Sträussler–Scheinker Italian family", Can J Neurol Sci 30 (3): 233–6. PMID 12945948</ref> due to inheritance. Onset of the disease usually occurs between the ages of 35 and 55. GSS syndrome belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs) or [[Prion\|prion]] diseases<ref>K Hsiao, C Cass, GD Schellenberg, A prion protein variant in a family with the telencephalic form of Gerstmann–Sträussler–Scheinker syndrome, Neurology, 41 (1991), pp. 681–684</ref>.<br>

	=== Causes ===		=== Causes ===

	GSS is one of few diseases that are caused by the transformation of prion proteins; often due to small changes in its [[Amino acid\|amino acid]] sequence at a particular [[Codon\|codon]].  A change in the amino acid sequence from [[Proline\|Proline]] (P) to [[Leucine\|leucine]] (L) on [[Codon\|codon]] 102 found in [[Chromosome 20\|chromosome 20]]<ref>L Goldfarb, P Brown, A Vrbovská, An insert mutation in the chromosome 20 amyloid precursor gene in a Gerstmann–Sträussler–Scheinker family, J Neurol Sci, 111 (1992), pp. 189–194</ref>, is observed in the prion protein gene (PRNP) of most affected individuals. From current knowledge, this genetic change is usually required for the development of GSS. Genetic testing allows for the [[Diagnosis\|diagnosis]] of this underlying genetic mutation. This involves a [[Blood\|blood]] and [[DNA\|DNA]] sample from a potential patient and examining it in order to attempt to detect this mutated gene at certain codons. If the genetic mutation is present, the patient will eventually be afflicted by GSS, and, due to the genetic or inheritance-based nature of the disease, the offspring of the patient have a much higher risk of inheriting the Proline to Leucine mutation, thus contracting GSS.<br>		GSS is one of few diseases that are caused by the transformation of prion proteins; often due to small changes in its [[Amino acid\|amino acid]] sequence at a particular [[Codon\|codon]].  A change in the amino acid sequence from [[Proline\|Proline]] (P) to [[Leucine\|leucine]] (L) on [[Codon\|codon]] 102 found in [[Chromosome 20\|chromosome 20]]<ref>L Goldfarb, P Brown, A Vrbovská, An insert mutation in the chromosome 20 amyloid precursor gene in a Gerstmann–Sträussler–Scheinker family, J Neurol Sci, 111 (1992), pp. 189–194</ref>, is observed in the prion protein gene (PRNP) of most affected individuals. From current knowledge, this genetic change is usually required for the development of GSS. Genetic testing allows for the [[Diagnosis\|diagnosis]] of this underlying genetic mutation. This involves a [[Blood\|blood]] and [[DNA\|DNA]] sample from a potential patient and examining it in order to attempt to detect this mutated gene at certain codons. If the genetic mutation is present, the patient will eventually be afflicted by GSS, and, due to the genetic or inheritance-based nature of the disease, the offspring of the patient have a much higher risk of inheriting the Proline to Leucine mutation, thus contracting GSS.<br>

	=== Treatment ===		=== Treatment ===

	There is no known cure available GSS, and there are no known treatments to slow the progression of the disease. However, therapies and medication are aimed at treating or slowing down and alleviating the symptoms, which will hopefully lead to an increased quality of life for the patient.<br>		There is no known cure available GSS, and there are no known treatments to slow the progression of the disease. However, therapies and medication are aimed at treating or slowing down and alleviating the symptoms, which will hopefully lead to an increased quality of life for the patient.<br>

	=== References ===		=== References ===

	<references /><br>		<references /><br>

150351065 at 19:24, 30 November 2015

2015-11-30T19:24:06Z

← Older revision		Revision as of 19:24, 30 November 2015
Line 1:		Line 1:
	Gerstmann-Straussler-Scheinker (GSS) syndrome is a neurodegenerative disorder<ref>1. National Institute of Neurological Disorders and Stroke (NINDS), Gerstmann-Straussler-Scheinker Disease Information Page, (2007),[Online], Available at: http://www.ninds.nih.gov/disorders/gss/gss.htm. Accessed 3/21/2008, (Last Accessed: 26/11/14)</ref> affecting the [[Brain\|brain]]. This leads to conditions such as ataxia and dementia, which includes common symptoms of memory loss and the loss of balance and coordination, respectively. The disease itself is almost always inherited and is very rare; only a few known cases are reported around the world, running down through families<ref>De Michele G, Pocchiari M, Petraroli R, et al., (August 2003), "Variable phenotype in a P102L Gerstmann–Sträussler–Scheinker Italian family", Can J Neurol Sci 30 (3): 233–6. PMID 12945948</ref> due to inheritance. Onset of the disease usually occurs between the ages of 35 and 55. GSS syndrome belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs) or prion diseases.<ref>K Hsiao, C Cass, GD Schellenberg, A prion protein variant in a family with the telencephalic form of Gerstmann–Sträussler–Scheinker syndrome, Neurology, 41 (1991), pp. 681–684</ref><br>		Gerstmann-Straussler-Scheinker (GSS) syndrome is a neurodegenerative disorder<ref>1. National Institute of Neurological Disorders and Stroke (NINDS), Gerstmann-Straussler-Scheinker Disease Information Page, (2007),[Online], Available at: http://www.ninds.nih.gov/disorders/gss/gss.htm. Accessed 3/21/2008, (Last Accessed: 26/11/14)</ref> affecting the [[Brain\|brain]]. This leads to conditions such as ataxia and dementia, which includes common symptoms of memory loss and the loss of balance and coordination, respectively. The disease itself is almost always inherited and is very rare; only a few known cases are reported around the world, running down through families<ref>De Michele G, Pocchiari M, Petraroli R, et al., (August 2003), "Variable phenotype in a P102L Gerstmann–Sträussler–Scheinker Italian family", Can J Neurol Sci 30 (3): 233–6. PMID 12945948</ref> due to inheritance. Onset of the disease usually occurs between the ages of 35 and 55. GSS syndrome belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs) or [[Prion\|prion]] diseases.<ref>K Hsiao, C Cass, GD Schellenberg, A prion protein variant in a family with the telencephalic form of Gerstmann–Sträussler–Scheinker syndrome, Neurology, 41 (1991), pp. 681–684</ref><br>

	=== Causes ===		=== Causes ===

	GSS is one of few diseases that are caused by the transformation of prion proteins; often due to small changes in its [[~~amino~~ acid\|amino acid]] sequence at a particular [[Codon\|codon]].  A change in the amino acid sequence from [[Proline\|Proline]] (P) to [[Leucine\|leucine]] (L) on [[~~codon~~\|codon]] 102 found in [[~~chromosome~~ 20\|chromosome 20]]<ref>L Goldfarb, P Brown, A Vrbovská, An insert mutation in the chromosome 20 amyloid precursor gene in a Gerstmann–Sträussler–Scheinker family, J Neurol Sci, 111 (1992), pp. 189–194</ref>, is observed in the prion protein gene (PRNP) of most affected individuals. From current knowledge, this genetic change is usually required for the development of GSS. Genetic testing allows for the [[Diagnosis\|diagnosis]] of this underlying genetic mutation. This involves a [[~~blood~~\|blood]] and [[DNA\|DNA]] sample from a potential patient and examining it in order to attempt to detect this mutated gene at certain codons. If the genetic mutation is present, the patient will eventually be afflicted by GSS, and, due to the genetic or inheritance-based nature of the disease, the offspring of the patient have a much higher risk of inheriting the Proline to Leucine mutation, thus contracting GSS.<br>		GSS is one of few diseases that are caused by the transformation of prion proteins; often due to small changes in its [[Amino acid\|amino acid]] sequence at a particular [[Codon\|codon]].  A change in the amino acid sequence from [[Proline\|Proline]] (P) to [[Leucine\|leucine]] (L) on [[Codon\|codon]] 102 found in [[Chromosome 20\|chromosome 20]]<ref>L Goldfarb, P Brown, A Vrbovská, An insert mutation in the chromosome 20 amyloid precursor gene in a Gerstmann–Sträussler–Scheinker family, J Neurol Sci, 111 (1992), pp. 189–194</ref>, is observed in the prion protein gene (PRNP) of most affected individuals. From current knowledge, this genetic change is usually required for the development of GSS. Genetic testing allows for the [[Diagnosis\|diagnosis]] of this underlying genetic mutation. This involves a [[Blood\|blood]] and [[DNA\|DNA]] sample from a potential patient and examining it in order to attempt to detect this mutated gene at certain codons. If the genetic mutation is present, the patient will eventually be afflicted by GSS, and, due to the genetic or inheritance-based nature of the disease, the offspring of the patient have a much higher risk of inheriting the Proline to Leucine mutation, thus contracting GSS.<br>

	=== Treatment ===		=== Treatment ===

Nnjm2 at 23:36, 26 November 2014

2014-11-26T23:36:30Z

← Older revision		Revision as of 23:36, 26 November 2014
Line 1:		Line 1:
	Gerstmann-Straussler-Scheinker (GSS) syndrome is a neurodegenerative disorder<ref>1. National Institute of Neurological Disorders and Stroke (NINDS), Gerstmann-Straussler-Scheinker Disease Information Page, (2007),[Online], Available at: http://www.ninds.nih.gov/disorders/gss/gss.htm. Accessed 3/21/2008, (Last Accessed: 26/11/14)</ref> affecting the [[Brain\|brain]]. This leads to conditions such as ataxia and dementia, which includes common symptoms of memory loss and the loss of balance and coordination, respectively. The disease itself is almost always inherited and is very rare; only a few known cases are reported around the world, running down through families<ref>De Michele G, Pocchiari M, Petraroli R, et al., (August 2003), "Variable phenotype in a P102L Gerstmann–Sträussler–Scheinker Italian family", Can J Neurol Sci 30 (3): 233–6. PMID 12945948</ref> due to inheritance. Onset of the disease usually occurs between the ages of 35 and 55. GSS syndrome belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs) or prion diseases.<ref>K Hsiao, C Cass, GD Schellenberg, A prion protein variant in a family with the telencephalic form of Gerstmann–Sträussler–Scheinker syndrome, Neurology, 41 (1991), pp. 681–684</ref>		Gerstmann-Straussler-Scheinker (GSS) syndrome is a neurodegenerative disorder<ref>1. National Institute of Neurological Disorders and Stroke (NINDS), Gerstmann-Straussler-Scheinker Disease Information Page, (2007),[Online], Available at: http://www.ninds.nih.gov/disorders/gss/gss.htm. Accessed 3/21/2008, (Last Accessed: 26/11/14)</ref> affecting the [[Brain\|brain]]. This leads to conditions such as ataxia and dementia, which includes common symptoms of memory loss and the loss of balance and coordination, respectively. The disease itself is almost always inherited and is very rare; only a few known cases are reported around the world, running down through families<ref>De Michele G, Pocchiari M, Petraroli R, et al., (August 2003), "Variable phenotype in a P102L Gerstmann–Sträussler–Scheinker Italian family", Can J Neurol Sci 30 (3): 233–6. PMID 12945948</ref> due to inheritance. Onset of the disease usually occurs between the ages of 35 and 55. GSS syndrome belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs) or prion diseases.<ref>K Hsiao, C Cass, GD Schellenberg, A prion protein variant in a family with the telencephalic form of Gerstmann–Sträussler–Scheinker syndrome, Neurology, 41 (1991), pp. 681–684</ref><br>

	~~ ~~		=== Causes ===

	~~= Causes =~~		GSS is one of few diseases that are caused by the transformation of prion proteins; often due to small changes in its [[amino acid\|amino acid]] sequence at a particular [[Codon\|codon]].  A change in the amino acid sequence from [[Proline\|Proline]] (P) to [[Leucine\|leucine]] (L) on [[codon\|codon]] 102 found in [[chromosome 20\|chromosome 20]]<ref>L Goldfarb, P Brown, A Vrbovská, An insert mutation in the chromosome 20 amyloid precursor gene in a Gerstmann–Sträussler–Scheinker family, J Neurol Sci, 111 (1992), pp. 189–194</ref>, is observed in the prion protein gene (PRNP) of most affected individuals. From current knowledge, this genetic change is usually required for the development of GSS. Genetic testing allows for the [[Diagnosis\|diagnosis]] of this underlying genetic mutation. This involves a [[blood\|blood]] and [[DNA\|DNA]] sample from a potential patient and examining it in order to attempt to detect this mutated gene at certain codons. If the genetic mutation is present, the patient will eventually be afflicted by GSS, and, due to the genetic or inheritance-based nature of the disease, the offspring of the patient have a much higher risk of inheriting the Proline to Leucine mutation, thus contracting GSS.<br>

	GSS is one of few diseases that are caused by the transformation of prion proteins; often due to small changes in its amino acid sequence at a particular [[Codon\|codon]].  A change in the amino acid sequence from [[Proline\|Proline ]](P) to [[Leucine\|leucine ]](L) on codon 102 found in chromosome 20<ref>L Goldfarb, P Brown, A Vrbovská, An insert mutation in the chromosome 20 amyloid precursor gene in a Gerstmann–Sträussler–Scheinker family, J Neurol Sci, 111 (1992), pp. 189–194</ref>, is observed in the prion protein gene (PRNP) of most affected individuals. From current knowledge, this genetic change is usually required for the development of GSS. Genetic testing allows for the [[diagnosis\|diagnosis]] of this underlying genetic mutation. This involves a blood and DNA sample from a potential patient and examining it in order to attempt to detect this mutated gene at certain codons. If the genetic mutation is present, the patient will eventually be afflicted by GSS, and, due to the genetic or inheritance-based nature of the disease, the offspring of the patient have a much higher risk of inheriting the Proline to Leucine mutation, thus contracting GSS.<br>		=== Treatment ===

	<br>		There is no known cure available GSS, and there are no known treatments to slow the progression of the disease. However, therapies and medication are aimed at treating or slowing down and alleviating the symptoms, which will hopefully lead to an increased quality of life for the patient.<br>

	= ~~Treatment~~ =		=== References ===

	There is no known cure available GSS, and there are no known treatments to slow the progression of the disease. However, therapies and medication are aimed at treating or slowing down and alleviating the symptoms, which will hopefully lead to an increased quality of life for the patient.		<references /><br>

	~~<br>~~

	~~= References =~~

	~~ ~~ <references />~~ ~~

	~~ ~~ <~~references /> ~~

	~~  <references /> ~~

	~~  <references /> ~~

	~~  <references /~~>

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Gerstmann-Straussler-Scheinker (GSS) syndrome is a neurodegenerative disorder<ref>1. National Institute of Neurological Disorders and Stroke (NINDS), Gerstmann-Straussler-Scheinker Disease Information Page, (2007),[Online], Available at: http://www.ninds.nih.gov/disorders/gss/gss.htm. Accessed 3/21/2008, (Last Accessed: 26/11/14)</ref> affecting the [[Brain|brain]]. This leads to conditions such as ataxia and dementia, which includes common symptoms of memory loss and the loss of balance and coordination, respectively. The disease itself is almost always inherited and is very rare; only a few known cases are reported around the world, running down through families<ref>De Michele G, Pocchiari M, Petraroli R, et al., (August 2003), "Variable phenotype in a P102L Gerstmann–Sträussler–Scheinker Italian family", Can J Neurol Sci 30 (3): 233–6. PMID 12945948</ref> due to inheritance. Onset of the disease usually occurs between the ages of 35 and 55. GSS syndrome belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs) or prion diseases.<ref>K Hsiao, C Cass, GD Schellenberg, A prion protein variant in a family with the telencephalic form of Gerstmann–Sträussler–Scheinker syndrome, Neurology, 41 (1991), pp. 681–684</ref>

 

= Causes =

GSS is one of few diseases that are caused by the transformation of prion proteins; often due to small changes in its amino acid sequence at a particular [[Codon|codon]].  A change in the amino acid sequence from [[Proline|Proline ]](P) to [[Leucine|leucine ]](L) on codon 102 found in chromosome 20<ref>L Goldfarb, P Brown, A Vrbovská, An insert mutation in the chromosome 20 amyloid precursor gene in a Gerstmann–Sträussler–Scheinker family, J Neurol Sci, 111 (1992), pp. 189–194</ref>, is observed in the prion protein gene (PRNP) of most affected individuals. From current knowledge, this genetic change is usually required for the development of GSS. Genetic testing allows for the [[diagnosis|diagnosis]] of this underlying genetic mutation. This involves a blood and DNA sample from a potential patient and examining it in order to attempt to detect this mutated gene at certain codons. If the genetic mutation is present, the patient will eventually be afflicted by GSS, and, due to the genetic or inheritance-based nature of the disease, the offspring of the patient have a much higher risk of inheriting the Proline to Leucine mutation, thus contracting GSS.<br>

<br>

= Treatment =

There is no known cure available GSS, and there are no known treatments to slow the progression of the disease. However, therapies and medication are aimed at treating or slowing down and alleviating the symptoms, which will hopefully lead to an increased quality of life for the patient.

<br>

= References =

  <references /> 

  <references /> 

  <references /> 

  <references /> 

  <references />