Progressive Supranuclear Palsy: Symptoms, Causes, and Treatment
Progressive supranuclear palsy is a chronic neurodegenerative disease of unknown origin. To date, it has no specific treatment.
It usually starts with mood changes, balance disturbances, and frequent falls. But it evolves to other severe symptoms, such as paralysis of the gaze and difficulty swallowing.
Survival is, on average, 4 to 7 years after diagnosis.
It’s commonly confused with Parkinson’s disease in its early stages. Another name for it is Steele Richardson Olszewski syndrome.
What is progressive supranuclear palsy?
Progressive supranuclear palsy is a chronic, multisystemic, neurodegenerative, progressive disease. The term neurodegenerative is used to describe damage to the central nervous system resulting from the death of neurons and glia.
On the other hand, progressive refers to the fact that the disease worsens over time. It is multisystemic, because it doesn’t affect a single body system.
Finally, the word paralysis refers to the inability to move the eye muscles, while supranuclear refers to the nuclei of the brain that control eye movement.
The onset of the disease is insidious, i.e. symptoms appear slowly. The natural course is about 4 to 7 years from the first signs.
The classic clinical symptoms are usually gait instability with frequent falls, cognitive impairment, and supranuclear gaze palsy. Postural imbalance, with frequent falls, results from neck and trunk stiffness. Prior to falls, people with progressive supranuclear palsy often manifest dizziness, which is mistaken for middle ear problems.
In the beginning, there are also personality changes, memory problems, and slowness of movement. In addition, cognitive impairment expands to thinking and speech.
As the disease progresses, there’s weakness in eye movements and an inability to look down or up. The eyes appear frozen in their sockets and the upper eyelids retract.
There’s also stiffness of the phonatory muscles and those related to swallowing. Thus, there’s weakness in the movements of the mouth, tongue, and throat.
In addition, other symptoms of the mental sphere are associated, such as:
- Marked impairment of working memory
- Slowness from thinking to the development of movements
It can be confused with Parkinson’s disease
The confusion with Parkinson’s disease occurs mainly at the beginning. However, they differ in that in progressive supranuclear palsy there’s practically absent or low amplitude tremor. In addition, it’s postural and not at rest.
On the other hand, in Parkinson’s disease, the characteristic paralysis of the ocular muscles doesn’t occur. These elements serve for clinical differentiation.
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Progressive supranuclear palsy has no defined cause
An incidence of 4 cases per million people has been described. Its specific origin is unknown.
The degeneration is concentrated in the substantia nigra, the basal ganglia, the subthalamic nucleus, and the substantia reticularis. It’s established that this degeneration is explained by the abnormal accumulation of tau protein.
This protein is important for the health of brain cells, because it maintains and stabilizes microtubules, which are structures that allow the movement of neurons. When it accumulates in excess, the substance clumps together and causes cell death.
There are no known risk factors for progressive supranuclear palsy.
Although there’s no risk factor, it’s more common in males and after 50 years of age. It has a peak incidence after the age of 60 years.
The definitive diagnosis is made by studying the affected tissue by pathological anatomy. Unfortunately, this is a study that is only possible post mortem, that is, when the patient has already died. This examination reveals the accumulation and aggregation of tau protein.
There’s no effective treatment
Currently, there’s no specific treatment for progressive supranuclear palsy. Symptoms often don’t respond to drugs.
Some drugs identical to those prescribed for Parkinson’s disease are often used. Because there is a loss of dopamine-producing neurons, patients may benefit from the use of L-DOPA.
However, the effectiveness of this approach is not so good and response decreases over time. This is why lisuride has recently been included as an option, as it works by mimicking dopamine and serotonin.
Mental-related symptoms are usually managed with tricyclic antidepressants. For dementia, amphetamines and phenothiazines are considered.
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Physical therapy should be included to help improve joint stiffness and mobility—also occupational therapy to improve balance. Speech therapy approaches are directed at language.
When the involvement of the swallowing muscles no longer allows eating, people with progressive supranuclear palsy may require a gastrostomy. A tube is inserted into the skin of the abdomen and directed into the stomach.
Injection of botulinum toxin into the muscles around the eyes can help with excessive eyelid closure. But this isn’t a curative treatment and has no permanent effects.
Progressive supranuclear palsy: rare but serious
People with progressive supranuclear palsy often die from respiratory complications, such as pneumonia. This is caused by incoordination of the muscles associated with swallowing, which allows aspiration of food or other substances (broncho-aspiration).
In addition, disabling fractures or head trauma may occur due to increased susceptibility to falls. The evolution becomes more torpid with these complications and the future prognosis is reduced.
For the time being, non-pharmacological therapies try to allow affected persons to lead a life as close to normal as possible. There remains the hope of finding a pharmacological mechanism to halt neuronal degeneration.It might interest you...