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Progressive supranuclear palsy: causes, symptoms and treatments

Progressive supranuclear palsy: causes, symptoms and treatments

March 28, 2024

It is well known that the nervous system, formed by the nerves, the spinal cord and the brain, is a structure that governs all the functions of the organism. However, when something fails in this system, begin to appear problems in the ability to move, in speech, and even in the ability to gobble or breathe.

More than 600 neurological disorders have been recorded. However, many of them still pose a mystery to the scientific community. One of these mysteries is progressive supranuclear palsy , which mainly affects the movement of the person, but from which the concrete causes or an effective remedy have not been established.


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What is progressive supranuclear palsy?

Progressive supranuclear palsy is considered as a strange neuronal disorder which interferes with a large number of functions that the person performs in his daily life. These tasks range from difficulties in movement, balance, verbal communication, ingestion of food and sight, to mood, behavior and reason.

As indicated by its own name, this disease causes weakening and paralysis of brain areas over the brain nuclei , hence it is dominated supranuclear and, in addition, it evolves in a degenerative way, making the person worse little by little.


Like many other diseases, progressive supranuclear palsy affects men more than women , being the risk of suffering it significantly greater after 60 years of age. Even so, this is a rare disease since only about 3 to 6 people in every 100,000 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 start suffering from balance losses for no apparent reason, suffering from falls, muscle hardening and walking problems.

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


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

  • Depression
  • Apathy
  • Alterations in the trial
  • Difficulty solving problems
  • Anomia
  • Emotional lability
  • Anhedonia

The aspects concerning speech they are also altered to a greater or lesser degree. Speech becomes a slow and unintelligible speech, accompanied by a lack of facial expression. Likewise, swallowing capacity is also affected, hindering the ability to swallow both solids and liquids.

Its causes

The specific origin that causes this type of paralysis has not yet been discovered, however, as a result of the examination of the symptoms, it is known that neuronal damage progresses gradually in the area of ​​the brainstem.

However, the most distinctive feature of this disorder is the agglomeration of abnormal deposits of TAU proteins in the brain cells, causing that these do not work properly and end up dying.

The accumulation of this TAU protein causes progressive supranuclear palsy to be included in the diseases taupathies , which encompass 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. Theory of propagation

This assumption places the cause of the disease in the propagation of the TAU protein by contacting 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, spreading like this throughout the entire nervous system .

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

2. Theory of free radicals

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

Although the body is programmed to get rid of these free radicals, it is conjectured that, under what conditions, free radicals may 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, it is it is quite complicated to diagnose . In addition, there is no concrete evidence for its diagnosis.

For a diagnosis as exhaustive as 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 in ruling 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. But nevertheless, antiparkinson medications can help in a certain degree to people with problems of physical stability, slowness and hardening of the muscles.

Recent studies direct their approach towards the possibility of eliminating agglomerated TAU protein. These studies have developed a compound that prevents the accumulation of TAU, but is still in the process of establishing the safety and tolerability of this.

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

As for the difficulties in swallowing, if these are aggravated to constitute 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 reaching the stomach, this being the only possible way in which the patient can feed.

Forecast

The prognosis for this type of paralysis is not too encouraging. The disease provokes that the health of a person 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.


Meet Neurologist Dr. Alessandro Di Rocco (March 2024).


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