Progressive Supranuclear Palsy

Overview of Progressive Supranuclear Palsy

Progressive Supranuclear Palsy (PSP) is a brain disorder that happens due to the deterioration of regions within the brain causing loss of eye and body movement, coordination, and balance. Supranuclear is basically the nuclei of the nerve cells that are damaged which results in an overall state of weakness, and palsy. 

About every 3-6 individuals in every 100,000 have PSP, making it much less common than Parkinson’s disease. The risk factor associated with PSP primarily has to do with one’s age mostly affecting people in the 60-plus age bracket. So far there is no cure to treat PSP except for treatment options to alleviate the symptoms pertaining to it.

Signs and Symptoms of Progressive Supranuclear Palsy

The symptoms associated with PSP vary from person to person, however, common signs are that people lose their balance often and have problems with mobility. They also have slow eye movement and difficulty in shifting their gaze vertically. Other possible symptoms are a change in behavior where one loses interest in otherwise engaging activities, difficulty in problem-solving situations, and showcasing impulsive behavior, as well as being irritable most of the time. These symptoms can worsen as the disorder progresses affecting speech delivery and loss of memory.

Symptoms of Parkinson’s Disease and PSP overlap quite a lot because of which PSP is often misdiagnosed with the former. The doctor, therefore, has to rule out other possible conditions that might be a cause of the symptoms before correctly diagnosing PSP. 

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Types of Progressive Supranuclear Palsy

There are five types of PPS based on differences in the severity of brain damage and the distribution of the damage. 

  1. Richardson’s Syndrome- Difficulty in vertical eye movement, postural instability, deterioration in cognitive abilities, and change in personality. 
  2. PSP-Parkinsonism- Limb rigidity and cognitive dysfunction
  3. PSP-Pure Akinesia with Gait Freezing- Freezing of gait and inability to move muscles voluntarily
  4. PSP-Corticobasal Syndrome- Uncontrollable muscle contraction and eye movement abnormality
  5. Progressive Non-Fluent Aphasia- Non Fluent in spontaneous speech

In case you exhibit any concerning signs and symptoms of PSP, consult a medical professional as soon as possible.

Causes of Progressive Supranuclear Palsy

The deposition of an abnormal amount of protein, tau, on the brain cells results in brain damage. Tau is normally present in the brain and is broken down if it is in excess. However, in people with PSP, tau is not broken down properly as a result which brings about its effect by damaging brain cells as it forms clumps within the cells. Substantia nigra is one of the regions where tau accumulates. This is also the region affected by Parkinson’s Disease and explains why there is an overlap of symptoms between the two conditions.

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Diagnosis

People suffering from PSP might become disabled 3-5 years from its onset. If PSP progresses through the years, symptoms might worsen leading to severe complications which include pneumonia, fracture, and head injury. Due to these complications, the lifespan of people with PSP is around 5 years after diagnosis. Progression of PSP can transition from an early stage, consisting of symptoms that can be treated, such as poor balance, mood changes, and slight visual problems, to a late stage, where the symptoms become beyond healable, severe visual problems, reduced consciousness, and acute infections.

Treatment of Progressive Supranuclear Palsy | When to Consult a Doctor

So far there is no effective treatment that could cure PSP overall. There are only interventions that can be targeted against the symptoms that appear to relieve them. For example, in case of loss of balance and mobility problems, levodopa can be effective but the effects are short-lasting. As for depression that follows due to PSP, the intake of antidepressants may be beneficial. Medication for eye movement such as botulinum toxin can be followed to relax eye muscles. Physiotherapy would be required to aid in body movement and balance maintenance. 

Therapeutic approaches when it comes to clearance of tau deposition are still under exploration. For instance, to check the safety of a compound that would prevent tau accumulation or explore tau imaging agents that would help in monitoring PSP progression and the effects of any introduced intervention on the condition.

There are therefore treatment options to slow down the progression of PSP only and not to completely stop it. 

In case of any concerning signs and symptoms, please reach out to a certified neurologist

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