Progressive supranuclear palsy is an uncommon neurological, degenerative disorder of the central nervous system which leads to severe problems with balance and movement of the body. It is a form of Parkinson's syndrome that eventually damages the brain nerve cell clusters also known as the nuclei. As the brain cells get damaged over time it reduces vision, speech, and swallowing abilities, besides causing impairment of voluntary eye movements. These chronic disabilities cause deterioration in the quality of life. Treatment of this ailment mainly focuses on relieving symptoms as there is no way it can be completely cured.

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What Causes PSP?

The cause of progressive supranuclear palsy is unknown but most likely the disease occurs when brain cells in certain parts of the brain are damaged because of a build-up of a protein called tau. Abnormalities in tau protein lead to damage in both cortical (the outer layer of the cerebrum) as well as subcortical areas of the brain. Tau protein is formed in the brain naturally and gets broken down gradually. However, when the person is suffering from this ailment, this protein does not break itself and slowly starts to form clumps in brain cells. The reason for this intrinsically disordered protein malfunction can be an infectious agent, a random genetic mutation, or a chemical present in the food, air, or water.

Risk Factors

A common risk factor for this illness is generally identified as age. Elderly people tend to get affected by PSP more as compared to people below forty. In rare cases, younger people can be affected as well. The disease also affects men more than women. Moreover, environmental factors such as air pollution and pesticides and industrial solvents could increase the risk of this neurological, degenerative disorder

Symptoms Of PSP

The following are some of the commonly found symptoms of progressive supranuclear Palsy:

• Issues with mobility leading to loss of balance
• Sudden change in behavior patterns and irritability
• Muscle stiffness
• Extreme fatigue
• Reduced blinking reflex
• Poor judgment
• Inability to control eye and eyelid movement and blurred vision
• Slurred speech
• Memory loss
• Photophobia or aversion to bright light
• Disturbed sleep
• Neck pain and frequent bouts of headache
• Difficulty in swallowing or dysphagia

Also Read: Dysphagia: Causes, Symptoms And Treatment

Advanced PSP Stages

As PSP progresses to an advanced stage, people with the condition begin to lose their ability in controlling the muscles of their mouth, throat, and tongue which causes speech to become increasingly slurred. Some patients develop problems with thinking and concentration and in severe cases, some even get affected by dementia. Since the loss of control of throat muscles leads to swallowing problems, a feeding tube becomes essential after a certain point to prevent choking. There can be a possibility of chest infections as swallowing problems may lead to small food particles or fluid getting into the lungs. At advanced stages, bowels and bladder functions also become impaired. Constipation and difficulty passing urine are also commonly noticed. Some patients may also lose control over their bladder or bowel movements completely.

Diagnosis

Diagnosing progressive supranuclear palsy can be tricky because there are no specific laboratory tests or imaging techniques that can rule out this disorder. A diagnosis is generally done by evaluating the patient history besides doing some neurological scans. Some tests are done based on the pattern of the patient’s symptoms. The tests check the abilities such as memory, concentration, understanding of language and neuropsychological testing, and mental abilities. The test also rules out conditions that can cause similar symptoms, such as Parkinson’s disease or the possibility of stroke.

Treatment And Prevention For PSP

Presently, there is no cure or medicines for progressive supranuclear palsy, but a lot of therapies are recommended to help manage the symptoms of this disease. People in the early stages of the condition may get benefitted from taking medications used to treat Parkinson's disease or to improve balance, immobility, and joint stiffness. Regular exercises are also suggested that may help strengthen the muscles and improve posture and prevent joint stiffening. Speech therapies can also help to reduce swallowing problems and improve the overall quality of speech.