Is Progressive Supranuclear Palsy Inherited & Is It A Form Of Dementia?

Progressive supranuclear Palsy is generally not due to hereditary however rare cases of PSP can be inherited.1

The genetic cause of the disorder remains unknown but medical experts believe that PSP may be caused by a genetic mutation called MAPT.2

PSP typically affects people who are over 60 and causes dementia along with problems associated with movement.3,4

Progressive Supranuclear Palsy (PSP) is a rare progressive neurodegenerative disease caused by an overproduction of tau protein in brain cells resulting in damage in most important areas of the brain. It is characterized by disorders of problems and gait.

Case-control studies have been conducted on patients with PSP who had close relatives with Parkinson which shows strong evidence that it can be caused by a genetic disorder. There is currently no effective treatment for this condition however early intervention has provided beneficial results.

Is Progressive Supranuclear Palsy Inherited?

There is no known cause of the disease and is still under investigation. The disorder occurs due to the gradual disorientation of brain cells caused by a toxic buildup of misfolded tau proteins affecting balance, movement, vision, speech, and swallowing.

In very rare instances, progressive supranuclear palsy develops within the family. In a nutshell, progressive supranuclear palsy is not due to hereditary however rare cases of PSP can be inherited. However, the medical link is not clearly stated in clinical theories.1

Medical studies show people with progressive supranuclear palsy have close associates with dementia and Alzheimer’s. The genetic cause of the disorder remains unknown but medical experts believe that PSP may be caused by a genetic mutation called MAPT (microtubule-associated protein tau). These proteins predominantly present in neuron cells than in non-neuron to modulate the stability of axonal microtubules.

When MAPT mutation is inherited in a pattern of inheritance (autosomal dominant manner) meaning it produces one copy of the gene which is more than enough to instigate the condition in the individual. In certain instances, the individual mutates the gene from the affected parent which eventually results in higher chances of getting the condition. However, in sporadic cases, it can even make its occurrence in individuals who have no history of progressive supranuclear palsy.2

Is Progressive Supranuclear Palsy A Form Of Dementia?

PSP typically affects people who are over 60 and causes dementia along with problems associated with movement. It has the potential to cause several other diseases such as Parkinson’s dementia, and Alzheimer’s.

Medical studies demonstrate that one in 10 people affected with this condition encounter problem related to thinking and perception when your healthcare provider diagnosis the disorder. Most people who experience progressive supranuclear palsy will have dementia during some point of their lifetime.

Dementia is a set of symptoms affecting memory, thinking, and social abilities that often interfere with your daily life. Although it is a normal part of aging, yet progressive supranuclear palsy patients will experience severe symptoms of dementia as their stage progresses. On several occasions, these individuals will show changes in their behavior and emotions which is a typical symptom of frontotemporal dementia.

Change in behaviors often results in embarrassing moments in public places and social gatherings because patients with progressive supranuclear palsy lose their ability to control their emotions. They do prolonged crying and appear indifferent towards their family, friends, and other people.

However, changes in mood and behavior can also be due to depression so clinical studies show that progressive supranuclear palsy symptoms are quite overlapping, and diagnosis seems challenging.3,4

References:

  1. “Progressive Supranuclear Palsy.” Mayo Clinic, Mayo Foundation for Medical Education and Research, 7 Feb. 2020, www.mayoclinic.org/diseases-conditions/progressive-supranuclear-palsy/symptoms-causes/syc-20355659.
  2. “Progressive Supranuclear Palsy.” Genetic and Rare Diseases Information Center, U.S. Department of Health and Human Services, rarediseases.info.nih.gov/diseases/7471/progressive-supranuclear-palsy.
  3. “Progressive Supranuclear Palsy Fact Sheet.” National Institute of Neurological Disorders and Stroke, U.S. Department of Health and Human Services, www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Progressive-Supranuclear-Palsy-Fact-Sheet.
  4. “Dementia – Other Forms of Dementia – Neurodegenerative Diseases – Progressive Supranuclear Palsy (PSP).” Alzheimer Europe, www.alzheimer-europe.org/Dementia/Other-forms-of-dementia/Neurodegenerative-diseases/Progressive-Supranuclear-Palsy-PSP.

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