Progressive supranuclear palsy (PSP) is a rare brain disorder that affects almost every aspect of a person's function. It can impair thinking, speech, general communication, and physical movement. The exact cause of PSP is not currently known, but there are several theories. There are also specific risk factors that can cause an individual to have a higher chance of developing progressive supranuclear palsy. Progressive supranuclear palsy often presents with similar symptoms to Parkinson's disease, but there are differences between the two. For one, PSP is caused by a different neurological process than Parkinson's. However, initial misdiagnosis of progressive supranuclear palsy as Parkinson's is frequent, especially given how much more widespread Parkinson's disease is. These are the main risk factors, potential causes, and complications of progressive supranuclear palsy.
Abnormal Amounts of Tau
The main defining factor of progressive supranuclear palsy is an accumulation of abnormal amounts of tau, a protein that occurs naturally within the brain. However, in PSP, the ability to process the protein properly erodes, causing an excess to build up. These protein deposits affect the brain's nerve cells, interrupting and damaging them.
Tau is a key cause of other disorders as well. The group of tau-related disorders is called tauopathies. Other diseases and disorders related to tau accumulation are certain types of frontotemporal degeneration, corticobasal generation, and Alzheimer's disease. To treat the disease itself, scientists would need to find a way to keep tau from clumping harmfully. Right now, there is no treatment for PSP itself, but there are treatments and ways of managing the symptoms as they progress.
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Deterioration Of Brain Cells
The more the tau deposits accumulate, the more a person will experience deterioration of brain cells. This disease is progressive, meaning it continually worsens over time. The exact symptoms individuals experience will vary depending on the location of the tau deposits. Different areas of the brain affect different aspects of the mind and body.
As the brain cells continue to deteriorate, patients will experience any number of neurological symptoms. They might have difficulty moving their eyes and walking. Overall, their motor function might seem stiff and awkward, and there are often speech impairments. This covers the inability to speak at an average rate without pauses, but it also pertains to the inability to understand or put together sentences.
Another part of brain cell deterioration is a lack of emotional control or awareness. Individuals may swing wildly from one mood to the next with no apparent reason for the switch. They may also lash out in anger or become withdrawn and mask-like. The exact emotional behaviors will vary depending on the type of destruction done to the brain, along with the severity of that destruction.
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Age is the main risk factor researchers have found for progressive supranuclear palsy. It is rare for PSP to develop in any individuals under the sixty, and symptoms usually manifest for the first time in a person's early 60s. That said, there have been cases in which symptoms manifested in people who were still in their 40s or 50s. However, there have not been known cases of the disease occurring in children or young adults.
PSP is a rare disease, and because of this, not a lot is known about the risk factors. It seems it affects men more than women, but there's no conclusive evidence regarding whether this is coincidence or because of scientific reason. About twenty thousand patients have received a PSP diagnosis. However, it's possible the rate of occurrence is higher, and that many cases have been misdiagnosed as Parkinson's disease.
Continue reading to reveal if there is a genetic link to progressive supranuclear palsy.
Lack of Genetic Link
There have been rare cases of PSP in which the disease runs in families. Usually, though, cases are isolated incidents rather than cases implying a genetic link. Researchers have failed to uncover any links between progressive supranuclear palsy and a person's lifestyle, occupation, and surroundings. Right now, research is being conducted to find out whether certain genes predispose people to PSP development. The current evidence implies there is a lack of a genetic link.
In rare cases, two people in a family will receive the same diagnosis. But there are not clear or proven genetic links researchers have found. The best way to distinguish between cerebral issues like progressive supranuclear palsy is to use neuroimaging methods rather than family history. If there is a genetic cause of the tau protein abnormality, researchers have not yet isolated the affected gene.
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Current Theories on Causes
Researchers understand the basic cause of the disease. Tau is a protein that occurs naturally within a person's brain, and the average person can process and dispose of tau. But some people lack the ability to process their tau properly, leading to buildups of tau in the brain, which cause the brain cells to degenerate. Not only is tau the main factor causing PSP, but it is also a primary concern with Alzheimer's. The frustrating part is researchers do not yet understand what causes a person to stop being able to process tau. If more was known about the tau processing system, scientists might be able to make artificial medications to process the tau buildups. This would be a method similar to giving diabetic people insulin.
Removal of the excess tau is the most important aspect of potential treatment. The patient should also have a way to keep tau buildup from forming in the future. However, scientists have yet to develop a therapy capable of managing this.
Current theories on causes hypothesize the lack of tau processing capability is most likely related to aging, given that tau-related illnesses almost always occur in older adults. But exactly why do we lose our ability to process tau as we get older? What system or ability is shutting down in our brains? When scientists have the answers to these questions, they may have more definitive answers about treatment options.
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One of the top causes of death for individuals affected by progressive supranuclear palsy is pneumonia caused by the aspiration of food particles or fluid. A progressive supranuclear palsy patient is likely to experience problems with their ability to swallow food and fluids properly. This impairment puts them at a greater risk of experiencing aspiration and subsequent colonization of bacteria in their lungs. When bacteria colonize in an affected individual's lungs, they have developed pneumonia. Symptoms of progressive supranuclear palsy-precipitated pneumonia include a high fever, pain in the chest, fatigue, blue tint to the skin, wheezing, shortness of breath, and a productive cough. The majority of individuals affected by progressive supranuclear palsy are already frail and vulnerable to illness. This predisposition to illness means recurrent bouts of pneumonia from aspiration can be deadly in an affected individual.
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Aspiration is a term used to describe when an individual accidentally inhales a foreign object into their windpipe, airways, and lungs. Aspiration is typically caused by food particles, fluids, saliva, water, stomach contents, smoke, dust, or fumes. When a foreign object is aspirated into the lungs, it can cause partial obstruction of the airway and problems with breathing. The most common causes of aspiration in progressive supranuclear palsy patients are a lack of control of the tongue and an impaired swallowing reflex. Dysphagia describes issues with swallowing due to an underlying problem or structural defect. Dysphagia is common in neurological diseases like dementia and progressive supranuclear palsy because the brain can no longer communicate with the nerves responsible for moving the muscles that produce swallowing. Aspiration increases an individual's risk of developing pneumonia.
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Problems with initiating and maintaining a restful sleep are common among individuals with neurodegenerative disorders, like Alzheimer's disease, Parkinson's disease, and progressive supranuclear palsy. The problems with sleep in affected individuals have an association with the neuroanatomical parts of the brain affected by the degeneration that occurs in progressive supranuclear palsy being the same parts of the brain that are responsible for housing the individual's sleep/wake regulation system. On average, it can take an individual with progressive supranuclear palsy over an hour to fall asleep compared to between ten and twenty minutes for a healthy individual. Progressive supranuclear palsy patients are known to wake during the night more frequently than healthy individuals. Comorbidities like sleep apnea and restless leg syndrome are also common in individuals who have progressive supranuclear palsy, which can also contribute to difficulties with sleep.
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Increased Risk Of Injuries
One of the earliest symptoms reported in progressive supranuclear palsy is a loss of balance when they are trying to walk. The risk of injuries is increased in affected individuals because when they experience a fall, it tends to occur in the backward direction. Progressive supranuclear palsy patients also tend to have issues with their behavior and abnormally poor judgment skills. Some may act recklessly or take unnecessary risks. Some individuals affected by progressive supranuclear palsy may experience the manifestation of the involuntary movements of particular parts of their body, which can put them at an increased risk of injury when they attempt to use tools, scissors, utensils, and other objects. Blurry or double vision in an affected individual due to their disorder can put them at a higher risk of injury if they attempt to operate machinery or drive a vehicle.
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Trouble Looking At Lights
The ocular and visual symptoms that present in affected individuals are the main distinguishing factor of this disorder, as Parkinson's disease patients have many of the same symptoms. There are several reasons why an affected individual may have trouble looking at lights, including the impaired ability to move the eyelids at the proper times. Excessive dryness due to a reduced rate of blinking of the eyes can cause an individual with progressive supranuclear palsy to be extra sensitive to lights. A progressive supranuclear palsy patient can experience problems with looking at lights after they have developed an increased sensitivity due to problems with gaze control. When an affected individual cannot shift their gaze out of bright lights properly, they may sustain damage to the parts of the eye responsible for adjustments to brighter lighting conditions.