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Progressive supranuclear palsy

Progressive supranuclear palsy (PSP) is a rare brain disorder that causes serious and permanent problems with control of gait and balance, along with complex eye movement and thinking problems. It is not frequent (20 times less common than Parkinson's disease, a principal degenerative movement disorder) and affects elderly persons, especially men.

It is known that the symptoms of PSP are caused by the progressive deterioration of some of the brain neurons that control movement, similar to what happens in Parkinson's, the similarity of which makes it difficult to diagnose. What is not completely known is what makes these brain cells degenerate.

PSP worsens progressively, and although in itself is not life-threatening, it does predispose the patient to serious complications such as pneumonia, difficulties swallowing, falls and fractures. With good attention to medical and nutritional needs, it is possible for individuals with PSP to live a decade or more after the first symptoms of the disease and many patients live to past 70 years of age.

Symptoms

The disease begins and progresses slowly and the most frequent first symptom of PSP is a loss of balance while walking. Individuals may have unexplained falls or a stiffness and awkwardness in gait. However, the first definitive clue of PSP is the inability to control eye movements adequately due to lesions in those areas of the brain that coordinate them, resulting in a blurring of vision and problems controlling eye movement.

People with PSP often show alterations of mood and behavior, including depression and apathy. They lose interest in activities once enjoyed and show increased irritability and forgetfulness. They may also exhibit a slight progressive dementia.

Prevention

Given that the causes nor the triggers of the disease are known, there are no available effective medical measures to prevent PSP development. Elderly persons who show some of the symptoms should see their doctor.

Gene or region studied

  • MAPT
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