"They say it is a curse imposed" - Residents of a remote Aboriginal community in Australia have suffered and died for many years from a mysterious disease that they cannot yet overcome.
This group of Aboriginal people lives on the small island of Groote Eiland, which is 50 km from the Northern Territories and most of the population suffers from a mysterious disease, which was given the name Machado-Joseph Disease.
It all starts with problems with walking and gradually the person becomes unable to walk. Then there are problems with swallowing food and defecation. Symptoms build up slowly but relentlessly and always lead to death.
Due to similar problems with lack of muscle control, this disease is often confused with Parkinson's syndrome. However, this is a completely different disease.
In total, 654 aborigines live on Groot Eiland and at least 100 of them are sick.
Scientifically, this ailment is called type 3 spinocerebellar ataxia, and all over the world it occurs in isolated cases in different countries, including Belgium, Guiana, Algeria, Brazil, France, Japan, and the USA. Moreover, a single center for the occurrence of this disease has not yet been identified; four different sources are known.
However, for some unknown reason, the highest number of people suffering from this disease lives in Groot Eiland.
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The type of disease that the aborigines suffer from came to them most likely from the Azores Islands of Flores and São Miguel, where it was discovered in 1972. And he got there presumably from the north of Portugal from the community of Sephardi Jews.
For Gayangwa Lalara, who is over 70 years old, this disease has long become a part of her life. Machado-Joseph's illness affected her father and all six of her siblings. The fact is that the disease is hereditary and can pass from parents to children.
One of Lalara's nephews died of the disease in 2014.
Machado-Joseph disease can affect people of all ages from 10-year-olds to 70-year-olds. And if a child is diagnosed with a severe illness, then he is unlikely to live more than 35 years. In addition to leg problems and muscle atrophy, patients have decreased visual acuity and may lose their ability to distinguish colors.
There is no cure for Machado Joseph Disease. To make life easier for patients, they only recommend physiotherapy and glasses.
Machado-Joseph disease occurs due to an abnormal protein in the brain caused by a faulty chromosome. This protein causes early death of nerve cells in a part of the brain called the cerebellum. The damage to the cerebellum initially causes muscle weakness and progresses over time to complete lack of control and permanent physical disability.
On the left is the damaged cerebellum, on the right it is still normal.
Studies show that the effects of the disease manifest themselves earlier in age with each new generation. Libby Messi, head of the Machado-Joseph Disease Foundation, says the number of sick children is on the rise and nothing can be done about it yet.
In the last stages of the disease, a person can not only move, but also speak, but remains fully conscious. Death most often occurs due to lung problems.
