Progressive Supranuclear Palsy: Causes, Symptoms And Treatments
It is well known that the nervous system, made up of the nerves, the spinal cord and the brain, is a structure that governs all the functions of the body. However, when something goes wrong in this system, problems begin to appear in the ability to move, speak, and even the ability to swallow or breathe.
More than 600 neurological disorders have been recorded. However, many of them still remain 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 an effective remedy have not been established.
Progressive supranuclear palsy is considered to be a strange neuronal disorder which interferes with a large number of functions that the person performs in their daily life. These tasks range from difficulties in movement, balance, verbal communication, food intake and vision, to mood, behavior and reason.
As its name indicates, this disease causes weakening and paralysis of the areas of the brain above the cerebral nuclei hence it is dominated supranuclear and, furthermore, it evolves in a degenerative way, making the person worse little by little.
Like many other diseases, progressive supranuclear palsy It affects men more than women, the risk of suffering from it being significantly higher after 60 years of age. Even so, this is a rare disease since only approximately 3 to 6 people out of every 100,000 worldwide suffer from it.
Due to the large number of aspects that are affected by this disease, the symptoms that each person presents can be very varied. However, the majority of these people begin to suffer from loss of balance for no apparent reason, leading to falls, muscle hardening, and problems walking.
As the paralysis progresses, vision problems begin to appear. Specifically, these problems materialize in the form of cloudy, imprecise vision and difficulty controlling 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:
The 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, swallowing ability 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 gradually advances in the area of the brain stem.
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.
The accumulation of this TAU protein makes progressive supranuclear palsy included in tauopathies diseases which encompass other disorders such as Alzheimer’s.
Since the exact cause of this paralysis is not known, there are two theories that attempt to explain this phenomenon:
This assumption places the cause of the disease in the spread of the TAU protein through contact with 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 spreading throughout the entire nervous system
What this theory does not explain is that this alteration begins, with a possibility 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 attempts to explain the causes of this disorder, theorizes that this damage caused to cells is caused by free radicals. Free radicals are reactive particles that cells make during natural metabolism.
Although the body is programmed to get rid of these free radicals, it is conjectured that, under certain conditions, free radicals can interact with other molecules and deteriorate them.
Diagnosis
Because progressive supranuclear palsy shares a large number of symptoms with many other diseases that affect movement, this It is quite complicated to diagnose Furthermore, there are no concrete tests for its diagnosis.
For the most comprehensive diagnosis possible, the clinician should be based on clinical history and a physical and neurological evaluation of the patient. In addition, diagnostic imaging tests, such as MRI or positron emission tomography (PET), can be of great help in ruling out other similar diseases.
Treatment
At the moment, no treatment has been found capable of curing progressive supranuclear palsy, although research is being carried out in methods to control the symptoms of the disease
Generally, the manifestations of this paralysis are not improved with any medication. However, antiparkinsonian medications can help to a certain extent to people with problems of physical stability, slowness and hardening of the muscles.
Recent studies direct their focus towards the possibility of eliminating the agglomerated TAU protein. These studies have developed a compound that prevents the accumulation of TAU, but the safety and tolerability of this compound are still in the process of being established.
At the level of physical movement, the person You can use instruments to help you maintain 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 must undergo a gastrostomy ; which involves the clinician installing 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 progressively deteriorates acquiring the category of severe disability between three and five years after its onset and with the risk of death approximately ten years after the onset of symptoms.
