Progressive bulbar palsy (PBP) is a form ofmotor neuron disease (MND).
PBP is considered a phenotype ofamyotrophic lateral sclerosis (ALS).
This article discusses the symptoms, causes, treatment, and prognosis for those diagnosed with the condition.
Charday Penn / Getty Images
Charday Penn / Getty Images
Progressive Bulbar Palsy Symptoms
Themotor neuronsaffected by PBP are lower motor neurons.
These cells send signals from upper motor neurons to the brain to control muscle movements.
Risks Associated with PBP Symptoms
Causes
The exact cause of PBP is unknown.
The gene aids in making a protein that is found in neurons.
Researchers are not yet clear on how this specific gene expansion plays a role in developing PBP.
The same gene has beencorrelated with the developmentoffamilial ALS, which is an inherited version of the disorder.
Diagnosis
Diagnosing PBP is based on several aspects of medical testing.
Initially, health history and physical examination of symptoms will be done.
After that, diagnostic tests will be performed to determine a definitive diagnosis.
Treatment
There is no cure for PBP.
Treatment can only make living with the disorder more comfortable.
How Well Does Treatment Work?
People respond to treatments differently, so its difficult to say what works for one will work for another.
Therapy can improve one’s quality of life but not eliminate the disease or its symptoms altogether.
Prognosis
PBP is a fatal disease.
Once a person is diagnosed, they will have to cope with it for the rest of their lives.
Similar to ALS, people with PBP have a drastically reducedlife expectancy.
However, research shows that this specific phenotype is associated with a shorter survival time.
Coping
Coping with a fatal disease is one of the hardest things a person will go through.
However, there are ways they can support themselves through it.
The exact cause of PBP isnt well understood, but researchers believe genetics may play a role.
Because medical scientists have yet to discover the source, there is no cure for the fatal disease.
People diagnosed with PBP can undergo treatments using medications, feeding tubes, or breathing aids.
However, those therapies are designed to improve a persons comfort and quality of life throughout their disease progression.
A Word from Verywell
Receiving a PBP diagnosis can turn your entire life upside down.
That said, it doesnt have to destroy the time you have left.
Give yourself time to grieve your situation and accept your fate.
Its all easier said than done but staying positive is the best way to handle a fatal diagnosis.
Frequently Asked Questions
Both progressive bulbar palsy and ALS are motor neuron disorders.
Motor neuron disorders affect the cells that send signals between the brain and the spinal cord.
Research has found that progressive bulbar palsy is likely a phenotype of ALS.
Once a person is diagnosed with progressive bulbar palsy, they will die from the disease.
When the symptoms begin, they may come on gradually but worsen over time.
Eventually, since there is no cure, those symptoms will cause health complications that lead to death.
People with progressive bulbar palsy develop symptoms that affect the facial muscles and tongue.
Because of that, it can be challenging to talk, eat, drink, and eventually breathe.
People with the disorder may feel unable to control their facial muscles.
USA Social Security Administration Program Operations Manual System.Progressive Bulbar Palsy.
