Gerstmann-Straussler-Scheinker Is Prion Disease

Gerstmann-Straussler-Scheinker disease (GSS) is a rare type of prion disease also known as transmissible spongiform encephalopathy (TSE). Prion diseases are a group of conditions that affect the nervous system. The main feature of GSS is a progressive degeneration of the cerebellum (a part of the brain that controls coordination, balance, equilibrium and muscle tone), as well as different degrees of dementia.

It is not easy to distinguish GSS from spinocerebellar ataxia, spastic paraparesis, frontotemporal dementia, Alzheimer’s disease, and Creutzfeldt-Jakob disease (CJD), particularly in the early stages of the disease.

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The symptoms, the progression of the disorder, and the overall severity can vary greatly among affected families and individuals. This is true even among members of the same family. A common symptom is a progressive loss of coordination that may present as unsteadiness of gait, difficulty walking, and clumsiness. As the disease progresses, other symptoms become apparent including dementia, in which there are worsening problems with thought, cognition, memory, language, and behavior. People with GSS have a 50 percent chance of passing the mutated gene on to their children. There is no known way to prevent GSS.

Treatment is based on the signs and symptoms, which vary from person to person. Signs and symptoms of Gerstmann-Straussler-Scheinker disease generally develop between the ages of 35 and 50 years. Affected people may experience:

  • Progressive ataxia, including clumsiness, unsteadiness, and difficulty walking;
  • Cognitive dysfunction;
  • Slurred speech;
  • Abnormal eye movements;
  • Rigid muscle tone;
  • Visual disturbances and blindness;
  • Difficulty swallowing;
  • Deafness; and

GSS is caused by an abnormal variant of the prion protein (PRPN) gene.

Parents have a 50 percent chance of passing the gene along to each child. Mutations in this gene spawn additional mutations of the prion protein, which are now toxic to the body. The abnormal prions build up primarily within the brain, which leads to the progressive loss of neurons. There is no cure or treatments to slow its progression.

On average, people affected by GSS survive approximately 60 months following diagnosis.

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