Thursday, June 14, 2018 by Janine Acero
Gerstmann-Straussler-Scheinker syndrome (GSS) is a rare familial form of progressive dementia that causes loss of coordination followed by slow deterioration of mental function. It usually begins earlier in life (affecting people in their 40s rather than in their 60s) and progresses more slowly (with an average life expectancy of five years rather than six months).
Gerstmann-Sträussler-Scheinker syndrome occurs as the result of a brain injury or as a developmental disorder. It is a prion disease that runs in families.
The deterioration of mental function usually starts in the fourth or fifth decade of life with gradual dysarthria (difficulty speaking) and cerebellar ataxia (wobbliness). The progressive dementia becomes evident later on. Death usually occurs within 10 years after the onset of symptoms.
The syndrome is a known form of transmissible spongiform encephalopathy (TSE). It refers to the characteristic plaques that form when infectious prions get into the brain – after people or animals eat food that is contaminated with them – which give the brain a “spongy” appearance.
The syndrome was first described in 1936 by the Austrian neurologists Josef Gerstmann (1887-1969), Ernst Sträussler (1872-1959), and I. Scheinker.
Gerstmann-Sträussler-Scheinker syndrome causes the loss of four specific neurological functions:
It is very rare for a person with learning disabilities to have all four of these mental dysfunctions.
A few cases have been reported in children, known as developmental Gerstmann syndrome. These cases usually become apparent when children begin school. Affected children may demonstrate poor handwriting, spelling and basic math skills. Some children have difficulty reading or understanding written words (alexia) and difficulty copying or tracing simple objects (constructional apraxia).
Symptoms of prion diseases include:
Complications that have been associated with prion diseases include:
There is no specific food or nutrient that may help prevent this rare prion disease. However, for humans, the best way to prevent spread during medical procedures is to get rid of any potentially contaminated surgical instruments; current sterilization procedures may not fully destroy prion proteins.
There is no specific treatment for prion diseases. The treatments are largely supportive. Treatments could be tailored to improve optimal brain function, as one might do for dementia.
If you are opting for the natural approach, a healthy diet and active lifestyle may ensure overall health. Your healthcare provider can make recommendations regarding proper nutrition, ensuring that adequate vitamins, minerals, and nutrients are consumed to avoid nutrient deficiencies.
Acupuncture has been known to effectively manage nerve and muscle problems. Herbs like Bacopa or Ginkgo could be used to see if any of the cognitive symptoms improve.
It’s always important to know where your meat comes from. It is recommended that humans consume organic meats with a verified source.
Gerstmann-Straussler-Scheinker syndrome is a rare hereditary form of progressive dementia that causes loss of coordination and slow deterioration of mental function. It usually occurs in people in their 40s and progresses more slowly, with an average life expectancy of five years.
Gerstmann-Sträussler-Scheinker syndrome is a prion disease that runs in families.
The deterioration of mental function usually starts in the fourth or fifth decade of life that causes difficulty speaking and wobbliness, among other symptoms. Death usually occurs within 10 years after the onset of symptoms.
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