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. The disease usually begins between the ages of 35 and 55.
In the early stages, people with GSS 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 people develop dementia.
Other symptoms may include
- Dysarthria (slurring of speech)
- Nystagmus (involuntary movements of the eyes)
- Spasticity (rigid muscle tone)
- 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 for GSS, nor are there any known treatments to slow progression of the disease. Current therapies are aimed at alleviating symptoms. GSS is a slowly progressive condition usually lasting from two to 10 years. The disease ultimately causes severe disability and finally death.
How can I or my loved one help improve care for people with Gerstmann-Straussler-Scheinker disease?
Consider participating in a clinical trial so clinicians and scientists can learn more about GSS and related disorders. Clinical research uses human volunteers to help researchers learn more about a disorder and perhaps find better ways to safely detect, treat, or prevent disease.
All types of volunteers are needed—those who are healthy or may have an illness or disease—of all different ages, sexes, races, and ethnicities to ensure that study results apply to as many people as possible, and that treatments will be safe and effective for everyone who will use them.
Where can I find more information about Gerstmann-Straussler-Scheinker disease?
Information also may be available from the following resources:
National Organization for Rare Disorders (NORD)
Phone: 203-744-0100 or 800-999-6673; 844-259-7178 Spanish