Progressive supranuclear palsy: symptoms and treatments

It is well known that the nervous system, made up of nerves, spinal cord and the brainIt is a structure that governs all the functions of the organism. However, when something goes wrong with this system, problems begin to appear in the ability to move, speak, and even swallow or breathe.

More than 600 neurological disorders have been registered. However, many of them are still a mystery to the scientific community. One of these mysteries is progressive supranuclear palsy., which mainly affects the movement of the person, but for which the specific causes and effective remedy have not been established.

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What is progressive supranuclear palsy?

Progressive supranuclear palsy is considered as a rare neural disorder which interferes in a large number of functions that the person performs in their daily life. These tasks range from difficulties with movement, balance, verbal communication, food intake, and vision, to mood, behavior, and reason.

As its name indicates, this disease causes the weakening and paralysis of the areas of the brain above the cerebral nucleiHence, supranuclear disease is dominated and, in addition, it evolves in a degenerative way, making the person gradually worsen.

Like many other diseases, progressive supranuclear palsy affects men more than women, with the risk of suffering from it being significantly higher after 60 years of age. Even so, this constitutes a rare disease since only approximately 3 to 6 people out of 100,000 worldwide suffer from it.

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Symptoms of progressive supranuclear palsy

Due to the large number of aspects that are affected by this disease, the symptoms that each person presents can be very varied. However, most of these people begin suffering from loss of balance for no apparent reason, eventually suffering falls, muscle hardening and problems walking.

As the paralysis progresses, vision problems begin to manifest. Specifically, these problems materialize in the form of cloudy, inaccurate vision and difficulty controlling eye movement, specifically to scroll the gaze from top to bottom.

Regarding the psychological aspects of these symptoms, patients affected by this disease often suffer from variations in behavior and mood. These changes can be specified in:

• Depression
• Apathy
• Disturbances in the trial
• Difficulty solving problems
• anomie
• Emotional lability
• Anhedonia

Aspects concerning speech They are also altered to a greater or lesser degree. Speech becomes slow, unintelligible speech, accompanied by a lack of facial expression. Likewise, the ability to swallow is also affected, making it difficult to swallow both solids and liquids.

its causes

The specific origin that causes this type of paralysis has not yet been discovered, however, following the examination of the symptoms, it is known that there is neuronal damage that progresses gradually in the area of ​​the trunk encephalic.

However, the most distinctive feature of this disorder is the agglomeration of abnormal deposits of TAU proteins in brain cells, causing them to not function properly and end up dying.

Clumping of this TAU protein makes progressive supranuclear palsy included in tauopathic diseases, which include other disorders such as Alzheimer's.

Since the exact cause of this paralysis is not known, there are two theories that try to explain this phenomenon:

1. propagation theory

This assumption locates the cause of the disease in the propagation of the TAU protein through the contact of altered cells. From here, it is theorized that once the TAU protein has accumulated in a cell, it can infect the cell to which it is connected, thus extending through the entire nervous system.

What this theory does not explain is that this alteration begins, one possibility being that a pathogenic element, hidden for a long period of time, begins to cause these effects in the person.

2. free radical theory

This second assumption that tries to explain the causes of this disorder, theorizes that this damage caused to cells is caused by free radicals. Free radicals are reactive particles made by cells during natural metabolism.

Although the body is programmed to rid itself of these free radicals, it is conjectured that under depending on what conditions, free radicals can interact with other molecules and deteriorate them

Diagnosis

Since progressive supranuclear palsy shares a large number of symptoms with many other diseases that affect movement, this it is quite difficult to diagnose. In addition, there are no specific tests for its diagnosis.

For the most comprehensive diagnosis possible, the clinician should be based on the clinical history and a physical and neurological evaluation of the patient. In addition, diagnostic imaging tests, such as magnetic resonance imaging or Positron emission tomography (PET) can be of great help to rule out other similar diseases.

Treatment

At the moment, no treatment capable of curing progressive supranuclear palsy has been found, although it is being investigated in methods to control the symptoms of the disease.

Generally, the manifestations of this paralysis are not improved with any medication. However, antiparkinsonian drugs can help to a certain degree to people with problems of physical stability, slowness and hardness of the muscles.

Recent studies direct their focus towards the possibility of removing the clumped TAU protein. These studies have developed a compound that prevents the accumulation of TAU, but the safety and tolerability of this is still in the process of being established.

At the level of physical movement, the person You can use instruments to help you maintain your balance.. As well as the use of specialized lenses to correct vision difficulties.

Regarding swallowing difficulties, if these worsen to the point of constituting a risk, it is possible that the person should undergo a gastrostomy; which implies that the clinician install a tube that crosses the skin of the abdomen until it reaches the stomach, this being the only possible way in which the patient can feed.

Forecast

The prognosis for this type of paralysis is not very encouraging. The disease causes that the person's health deteriorates progressively, acquiring the category of severe disability between three and five years after its onset and with the risk of death located approximately ten years after the onset of symptoms.