Progressive supranuclear paralysis: causes, symptoms, and treatments
It is well known that the nervous system, consisting of the nerves, the spinal cord and the brain, is a structure that governs all the functions of the organism. However, when something goes wrong in this system, problems begin to appear in the ability to move, in speech, and even in the ability to swallow or breathe.
More than 600 neurological disorders have been reported. However, many of them are still a mystery to the scientific community. One of these mysteries is progressive supranuclear palsy , which mainly affects the person’s movement, but for which neither concrete causes nor an effective remedy have been established.
What is progressive supranuclear palsy?
Progressive supranuclear palsy is considered to be a rare neurological disorder which interferes with a large number of functions a person performs in daily life. These tasks range from difficulties in movement, balance, verbal communication, food intake, and vision, to mood, behavior, and reasoning.
As its name indicates, this disease causes the weakening and paralysis of the areas of the brain above the cerebral nuclei , hence its supranuclear dominance and, moreover, it is evolving in a degenerative way, making the person worse little by little.
Like many other diseases, progressive supranuclear palsy affects men more than women and the risk of suffering from it is significantly higher after the age of 60. Even so, it is a rare disease since only approximately 3 to 6 people out of 100,000 worldwide suffer from it.
Symptoms of progressive supranuclear palsy
Due to the many aspects that are affected by this disease, the symptoms that each person presents can be very varied. However, most of these people begin by suffering loss of balance for no apparent reason, and go on to suffer falls, muscle stiffness and walking problems.
As the paralysis progresses, vision problems begin to appear. Specifically, these problems take the form of blurred and inaccurate vision and difficulty in controlling eye movement , specifically in shifting the gaze up and down.
As for the psychological aspects of these symptoms, patients affected by this disease often suffer from variations in behaviour and mood. These changes can be seen in:
- Impaired judgment
- Problem solving difficulty
- Emotional Ability
The aspects concerning speech are also altered to a greater or lesser extent. Speech becomes slow and unintelligible, accompanied by a lack of facial expression. The ability to swallow is also affected, making it difficult to swallow both solids and liquids.
The specific origin of this type of paralysis has not yet been discovered. However, following examination of the symptoms, it is known that there is gradually advancing neuronal damage 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 malfunction and eventually die.
The stacking of this TAU protein makes progressive supranuclear palsy included in taupathies , which encompasses 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:
1. Theory of propagation
This assumption places the cause of the disease in the spread of the TAU protein through contact with altered cells. 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 does not explain this theory 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. Free radical theory
This second assumption, which attempts to explain the causes of this disorder, theorizes that this damage 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 what conditions free radicals may interact with other molecules and deteriorate them.
Because progressive supranuclear palsy shares a large number of symptoms with many other diseases that affect movement, it is quite complicated to diagnose . Furthermore, there are no specific tests for its diagnosis.
For a diagnosis that is as thorough as possible, the clinician should base it on the medical history and a physical and neurological evaluation of the patient. In addition, diagnostic imaging tests, such as magnetic resonance or positron emission tomography (PET), can be of great help in ruling out other similar diseases.
At the moment, no treatment has been found that can cure progressive supranuclear palsy, although research is underway on methods of controlling the symptoms of the disease .
Generally, the manifestations of this paralysis are not improved by any medication. However, antiparkinsonian drugs can help to some degree people with problems of physical stability, slowness and hardening of the muscles.
Recent studies direct their focus towards the possibility of eliminating agglomerated TAU protein. These studies have developed a compound that prevents the accumulation of TAU, but it is still in the process of establishing the safety and tolerability of TAU.
On the level of physical movement, the person can use instruments that help him/her to keep his/her balance . As well as, the use of specialized lenses to correct the difficulties in vision.
As far as swallowing difficulties are concerned, if these become more serious and constitute a risk, the person may have to undergo a gastrostomy , which implies that the clinician installs a tube that goes through the skin of the abdomen to the stomach, which is the only possible way for the patient to feed himself.
The prognosis for this type of paralysis is not very encouraging. The disease causes the health of the person to progressively deteriorate , 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.