What Is Progressive Supranuclear Palsy?
Progressive supranuclear palsy (PSP) is a rare degenerative neurological condition that causes progressive impairment in walking and balance, as well as impaired eye movement, particularly while looking towards the downward side, abnormal muscle tone (rigidity), speech difficulties (dysarthria), and swallowing and eating difficulties (dysphagia).
The collapse of the nervous system, particularly the death of neurons and GIA (certain types of cells) in the brain, is referred to as neurodegenerative. Progressive suggests that the symptoms worsen over time, and palsy means that the eye motions are affected. The term supranuclear refers to brain injury that occurs above the eye-moving centers in the brain. Personality changes and cognitive impairment are common among those who are affected. Symptoms usually appear after the age of 60; however, they might appear sooner.
Progressive supranuclear palsy's exact cause is uncertain. Parkinson's disease, Alzheimer's disease, corticobasal degeneration, and other neurodegenerative illnesses are frequently misdiagnosed as progressive supranuclear palsy.
Although progressive supranuclear palsy is not dangerous, symptoms intensify over time, and there is no cure. Complications emerge when symptoms increase and can be fatal.
What Is the Cause of Progressive Supranuclear Palsy?
The degeneration of brain cells in a few tiny but critical locations near the base of the brain causes progressive supranuclear palsy to develop. The substantia nigra is the most severely afflicted portion. Many of the palsy symptoms become more obvious when this section of the brain is impacted by the disease. The cause of brain cell degeneration is still being investigated. Progressive supranuclear palsy is a type of tauopathy (neurodegenerative disorder) in which aberrant phosphorylation of the protein tau is linked to the death of essential protein filaments in nerve cells. The etiology is unknown. The loss of nerve cells is thought to be caused by this neurofibrillary degeneration, and most experimental treatments are aimed at preventing tau disease.
Progressive supranuclear palsy is usually sporadic, which means it happens infrequently and has no identified cause. The condition is caused by mutations in the MAPT gene in just a few cases. This mutation gives the nerve cell incorrect instructions for producing tau. Most people have not been linked to genetic variables.
What Are the Symptoms of Progressive Supranuclear Palsy?
Movement, gait (walking) and balance, swallowing, speech, eye movements and vision, mood and behavior, and thinking are all affected by progressive supranuclear palsy. The pattern of indications and symptoms might vary greatly from one person to the next. A loss of balance while walking is the most common and early symptom of progressive supranuclear palsy. Individuals may have sudden and unexpected falls, as well as a stiff and awkward walk and delayed mobility.
Most people will experience blurred vision and issues managing eye movement as the condition advances. These signs and symptoms could include:
-
Slow eye movements.
-
Having difficulty moving the sight vertically downward or upward.
-
Problems with eyelid control.
-
Eyes are closed involuntarily.
-
Blinking that is prolonged or infrequent.
-
Opening eyes is difficult.
-
During a conversation, there is difficulty in maintaining eye contact.
Progressive supranuclear palsy (PSP) patients frequently experience mood and behavioral changes. These signs and symptoms could include:
-
Loss of interest in common enjoyable activities.
-
Changes in perception, insight, and problem-solving abilities.
-
Finding words is difficult for familiar things.
-
Apathy.
-
Irritability and forgetfulness.
-
For no underlying reason, one may find themselves smiling, weeping, or having aggressive outbursts.
Other signs and symptoms include:
-
Memory issues.
-
Speech that is slowed, slurred, or monotone.
-
Faces that have mask-like expressions.
-
Slow thoughts.
-
Swallowing meals or drinks is difficult.
How to Diagnose Progressive Supranuclear Palsy?
Progressive supranuclear palsy is diagnosed based on clinical indications and exam findings; however, it might be difficult to detect. There is no one test that can definitively point to it. It is frequently confused with Parkinson's disease (PD), and distinguishing progressive supranuclear palsy from Parkinson’s disease can take years.
If the healthcare professional suspects progressive supranuclear palsy based on the symptoms, he or she will most likely refer patients for magnetic resonance imaging (MRI). This will help to eliminate other diseases like Parkinson's, and it may occasionally reveal midbrain shrinkage, which raises the probability of progressive supranuclear palsy in the right clinical scenario. Patients will also be referred to a neurologist who specializes in movement disorders and Parkinson's disease.
Progressive supranuclear palsy has a variety of symptoms, but the one that establishes the diagnosis is difficulty moving the eyes up and down. Other common symptoms of progressive supranuclear palsy include falling and difficulty swallowing.
What Is the Treatment for Progressive Supranuclear Palsy?
-
The treatment for persons suspected of having progressive supranuclear palsy remains symptomatic and supportive, with continuing clinical studies attempting to uncover disease-modifying treatments, which often target the underlying tau pathology. Progressive supranuclear palsy does not have any viable medicinal or surgical treatments.
-
Despite its parkinsonian appearance, pharmacological agents have minimal long-term benefits for this disease. Levodopa can cause transitory bradykinesia (slowness of movement) and rigidity of gait, and Botulinum toxin can reduce involuntary eye shutting and has no long-term consequences.
-
Medications are used to treat clinical depression. They are occasionally used even if there is no proper diagnosis of depression because they can help with general resilience and the ability to manage. The most regularly given antidepressants are Fluoxetine and Amitriptyline.
-
Assistive walking devices that can be used for the prevention of falls are examples of non-drug treatments for progressive supranuclear palsy, which also often include counterweights for extensor postures. Prism glasses are commonly given to alleviate a patient's impaired ability to gaze down, allowing for greater visual stimuli for balance and walking. Exercise through physiotherapy has been shown to maintain strength, balance, and coordination in such patients.
-
As progressive supranuclear palsy develops, one may lose their ability to swallow completely, preventing them from eating or drinking. The healthcare practitioner may advise the patient to obtain a gastrostomy. A gastrostomy is a technique in which a tube is inserted into the stomach through the belly. The tube will deliver meals, drugs, and beverages to the body.
Conclusion:
Even though there is no cure, medications, and technologies that can help one manage symptoms, one can find strategies to make walking safer and improve eye vision with the help of the healthcare physician. Progressive supranuclear palsy is not lethal; however, it is critical not to inhale food particles (aspirate) while eating because this can be life-threatening. Although it is tempting to dismiss early symptoms as being clumsy or having an ear infection, it is important to consult a doctor as soon as possible, especially if someone is having difficulties with their eyes or vision.
