Bush-Gsell Disease

Bush-Gsell disease

Boucher-Gsell disease is a rare hereditary disorder characterized by progressive ataxia, retinal pigmentary degeneration and polyneuropathy.

The disease was first described by French physician Georges Boucher in 1922. In 1956, the Swiss doctor Otto Gsell added to the description of the symptoms and established the hereditary nature of the disease.

Main symptoms: progressive instability of gait and impaired coordination of movements due to damage to the cerebellum (ataxia), decreased vision up to blindness due to retinitis pigmentosa, sensory neuropathy.

The reason is mutations in the SACS or SIL1 genes. The disease is inherited in an autosomal recessive manner.

Treatment is symptomatic and supportive. The prognosis is poor, with an average life expectancy of approximately 58 years.



Boucher-Gsell disease is a rare disease that is characterized by inflammation of the mucous membrane of the stomach and duodenum. It was described by the French physician Boucher in 1869 and the Swiss physician Gsell in 1895.

Boucher-Gsell disease manifests itself in the form of abdominal pain, nausea, vomiting and diarrhea. It can lead to weight loss and dehydration.

Treatment of Bush-Gsell disease includes the use of antibiotics, anti-inflammatory drugs and other medications. However, in most cases, the disease requires surgical intervention.

Prevention of Bush-Gsell disease consists of following the rules of hygiene and a healthy diet. It is also recommended to undergo regular medical examinations and consult a doctor if symptoms of the disease appear.

Thus, bushel disease is a serious disease that requires timely treatment. Prevention and early diagnosis can help reduce the risk of developing this disease and improve the quality of life of patients.