Gerstmann Straussler scheincker

HI there ,
I am really loss for words right now I have recently lost my mother to this Rare Disease. I have seen my grandfather get sick and pass away and now my mother and now my aunt is coming down with this disease. I am really scared becuase this disease is very heridetary.Not alot of people have heard of it. This is the disease below.
What is Gerstmann-Straussler-Scheinker Disease?
Gerstmann-Straussler-Scheinker disease (GSS) is an extremely rare, neurodegenerative brain disorder. It is almost always inherited and is found in only a few families around the world. Onset of the disease usually occurs between the ages of 35 and 55. In the early stages, patients may experience varying levels of ataxia (lack of muscle coordination), including clumsiness, unsteadiness, and difficulty walking. As the disease progresses, the ataxia becomes more pronounced and most patients develop dementia. Other symptoms may include dysarthria (slurring of speech), nystagmus (involuntary movements of the eyes), spasticity (rigid muscle tone), and visual disturbances, sometimes leading to blindness. Deafness also can occur. In some families, parkinsonian features are present. GSS belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs). Other TSEs include Creutzfeldt-Jakob disease, kuru, and fatal familial insomnia.
There is no cure or anything out there for this disease and many people have the gene in my family. I do not know if I have it or not yet. Please any one knows anything come talk with me.

By sabrinawalston
Posted September 8, 2007 at 5:16 pm
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Hi Shasha,
My name is Sabrina, I am a 25 year old mother of 4, & I also have GSS. It has been in my family for many generations. I lost my father 11 years ago, he was 32. I also lost my uncle a year later, he was 31. I have been doing alot of research on my disease since my diagnosis 2 years ago. I also have lots of people in my family who currently have GSS, but we don't have much contact with them. My dad hadn't seen his father since he was 2, and that's where he got the gene. We had no clue about the disease until my dad was diagnosed. I am very interested in talking to someone who has had the same experiences. I am currently enrolled in a research program with UCSF.

By mothahen
Posted April 4, 2009 at 1:37 am
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Hi. I am just trying to find others who know what I have been going through. I lost my sister to GSS disease on Oct. 27, 2008. She was 44 years young. She started to not feel well when she had a late pregnancy at the age of 42. We finally realized she had the same disease my father died of at the age of 41. He was incorrectly diagnosed with Huntington's chorea. We are now sure that my grandfather and some of his brothers and sisters also died of the same thing. My sister was sent for an autopsy and we finally had a name for what has been plaguing my family for generations. Both my father and sister exhibited similar symptoms, but there were differences as well. I guess I just want to see if there are more symptoms to be aware of and just have others to talk to. This disease is so rare I feel quite lost. I have 2 brothers and we all have children so I guess you can see where my concerns lie. I hope to hear back from you all soon. Thank you for listening.

By majbaker
Posted June 21, 2009 at 6:28 am
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My husband has GSS and has had for the last 5 years. We are the Indiana Kindred. Iu med school does a lot of studys with this. My husband has been doing study for a long time even before he had problems. His mother and aunt both had it and we just lost his cousin 3 years ago. Both of our children got pasted over. They will never have it in there line again. His cousin was not so blessed his son does not have the gene but his dauther does. Hers is and early on set gene. My husbands was a late on set gene. we take it one day at a time and live it as if it was the last. With this thats all you can do.

By Southernbelljar
Posted September 29, 2009 at 7:12 pm
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Sabrina:
It has been 2 years since your post and I hope this finds you still healthy. My husband lost his wife to this disease in 2005 and he has 3 children who may or may not have it. I wanted to ask you - what made you want to get tested? Do you feel it has helped your life to know that you have GSS? My step-daughters are struggling with the decision to get tested and find out if they are positive. Your father died at a rather young age from this disease. My husband's first wife was 38 when she presented and 43 when she passed away. Has this early onset been common or a pattern in your family?
Anyway, I hope you are well and I hope to hear from you.

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