Gerstmann-Straussler-Scheinker Syndrome. Fatal family insomnia

Gerstman-Straussler-Scheinker syndrome is a hereditary prion pathology that bears a family character. Compared with Kreutzfeldt-Jakob disease, people of a younger age get sick (on average, Schlet earlier). 

The duration of the incubation period of Gerstman-Straussler-Scheinker syndrome varies between 5 ~ 30 years. Typical is the gradual loss of reflexes from the lower extremities, swallowing disorders, muscle hypotension, dysarthria, and dementia. The disease slowly progresses over 4-5 years and ends with the death of the patient. Diagnosis, treatment and prevention are similar to the measures used in the recognition and treatment of Kreutzfeldt-Jakob disease.  

Fatal Family Insomnia

Fatal familial insomnia is a hereditary prion disease described in 1986. as an autosomal dominant pathology and registered in people aged 25 to 70 years. The first manifestation is a progressive sleep disorder, accompanied by increased fatigue and not amenable to treatment. They are joined by arterial hypertension, tachycardia, constipation, impotence. 

Later, motor disorders (ataxia, dysarthria, convulsions, dystonic attacks) and circadian heart rhythm disturbances join them . The patient’s death occurs as a result of progressive pulmonary heart disease.  

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