Table of ContentsView AllTable of ContentsSymptomsCausesDiagnosisTreatmentPrognosisCopingFAQs
Table of ContentsView All
View All
Table of Contents
Symptoms
Causes
Diagnosis
Treatment
Prognosis
Coping
FAQs
Progressive bulbar palsy (PBP) is a form ofmotor neuron disease (MND). When people develop the condition, theneuronsthat communicate between the brain stem, spinal cord, and brain become damaged, causing symptoms like loss of speech and muscle twitching. PBP is considered a phenotype ofamyotrophic lateral sclerosis (ALS).
This article discusses the symptoms, causes, treatment, and prognosis for those diagnosed with the condition.
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Progressive Bulbar Palsy Symptoms
People with PBP may be at an increased risk of choking. They also have a higher chance of developing a specific type of lung infection known asaspiration pneumonia, which occurs when food, liquids, and bacteria enter the lower airways and lungs. Those with the disorder are also more likely to develop a type of dementia known asfrontotemporal dementiaand cognitive impairment.
Risks Associated with PBP SymptomsPeople with PBP may be at an increased risk of choking. They also have a higher chance of developing a specific type of lung infection known asaspiration pneumonia, which occurs when food, liquids, and bacteria enter the lower airways and lungs. Those with the disorder are also more likely to develop a type of dementia known asfrontotemporal dementiaand cognitive impairment.
Risks Associated with PBP Symptoms
The exact cause of PBP is unknown. That said,geneticsmay play a role, and research has shown that people with PBP have a type of genetic expansion that affects theC9orf72gene. 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.
Ongoing ResearchAs little is known about the cause of PBP, the National Center for Advancing Translational Sciences Genetic and Rare Diseases Information Center continues to conduct research surrounding PBP to gain a better understanding of the disorder.
Ongoing Research
As little is known about the cause of PBP, the National Center for Advancing Translational Sciences Genetic and Rare Diseases Information Center continues to conduct research surrounding PBP to gain a better understanding of the disorder.
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. They include:
Ruling Out Other ConditionsSince PBP presents with symptoms similar to many other disorders, it’s important to rule those out before developing a treatment plan. Along with neuroimaging and electrophysiological tests, aspinal tap or lumbar puncturemay be done to assess the health of the spinal fluid.
Ruling Out Other Conditions
Since PBP presents with symptoms similar to many other disorders, it’s important to rule those out before developing a treatment plan. Along with neuroimaging and electrophysiological tests, aspinal tap or lumbar puncturemay be done to assess the health of the spinal fluid.
There is no cure for PBP. Treatment can only make living with the disorder more comfortable. Therapies focus on alleviating symptoms and can include:
Riluzole, which is a medication designed to slow down nerve cell damage, may also be given to slow the progression of the disease.
How Well Does Treatment Work?People respond to treatments differently, so it’s 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.
How Well Does Treatment Work?
People respond to treatments differently, so it’s 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.
Experimental ALS Drug Slows Disease Progression in Preliminary Study
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.
People diagnosed with the disease will have an average survival rate of six months to three years after their symptoms begin.
Palliative Care and PBPOnce a person is diagnosed with PBP, they should begin to think aboutpalliative careimmediately. The symptoms progress quickly, so establishing a solid healthcare plan can make all the difference in a person’s quality of life.
Palliative Care and PBP
Once a person is diagnosed with PBP, they should begin to think aboutpalliative careimmediately. The symptoms progress quickly, so establishing a solid healthcare plan can make all the difference in a person’s quality of life.
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. Some coping techniques to improve quality of lifeinclude:
Staying Positive in the Face of PBPTo stay positive after your diagnosis, you can try the following:Spending quality time with the ones you love mostPractice gratitude and enjoy even the little things in lifeAllow yourself to grieve and feel negative emotions so you can let them go and focus on what’s important
Staying Positive in the Face of PBP
To stay positive after your diagnosis, you can try the following:Spending quality time with the ones you love mostPractice gratitude and enjoy even the little things in lifeAllow yourself to grieve and feel negative emotions so you can let them go and focus on what’s important
To stay positive after your diagnosis, you can try the following:
10 Practical Ways to Deal with Terminal Illness
Summary
PBP is a phenotype of ALS that involves lower motor neuron damage, leading to excessive drooling, difficulty swallowing and talking, muscle twitching, and weakness in the face and tongue.
The exact cause of PBP isn’t 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 person’s comfort and quality of life throughout their disease progression. People with PBP can expect to live anywhere from six months to three years after the onset of their symptoms. With a reduced life expectancy, focusing on the good things in life, such as family and loved ones, is essential.
A Word from Verywell
Receiving a PBP diagnosis can turn your entire life upside down. That said, it doesn’t have to destroy the time you have left. Give yourself time to grieve your situation and accept your fate.
That way, you can spend your remaining time with the people you love most, enjoy your life as much as possible, and be grateful for all the good moments you’ve experienced. It’s all easier said than done but staying positive is the best way to handle a fatal diagnosis.
Frequently Asked QuestionsBoth 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.Learn MoreBrain and Nervous System ConditionsOnce 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.Learn MoreThe Dying Process: What to ExpectPeople 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.Learn MoreUnderstanding the Spinal Cord and Motor Neuron Diseases
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.Learn MoreBrain and Nervous System Conditions
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.
Learn MoreBrain and Nervous System Conditions
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.Learn MoreThe Dying Process: What to Expect
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.
Learn MoreThe Dying Process: What to Expect
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.Learn MoreUnderstanding the Spinal Cord and Motor Neuron Diseases
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.
Learn MoreUnderstanding the Spinal Cord and Motor Neuron Diseases
5 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Zhang HG, Chen L, Tang L, Zhang N, Fan DS.Clinical Features of Isolated Bulbar Palsy of Amyotrophic Lateral Sclerosis in Chinese Population.Chin Med J (Engl).2017 Aug 5;130(15):1768-1772. doi:10.4103/0366-6999.211538National Center for Advancing Translational Science Genetic and Rare Diseases Information Center.Progressive Bulbar Palsy.USA Social Security Administration Program Operations Manual System.Progressive Bulbar Palsy.Bublitz SK, Weck C, Egger-Rainer A, Lex K, Paal P, Lorenzl S.Palliative Care Challenges of Patients With Progressive Bulbar Palsy: A Retrospective Case Series of 14 Patients.Front Neurol.2021 Sep 24;12:700103. doi:10.3389/fneur.2021.700103Hopwood V, Nanjing DA, Donnellan C.Motor Neurone Disease.Acupuncture in Neurological Conditions.2010:173-183. doi:10.1016/B978-0-7020-3020-8.00011-4
5 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Zhang HG, Chen L, Tang L, Zhang N, Fan DS.Clinical Features of Isolated Bulbar Palsy of Amyotrophic Lateral Sclerosis in Chinese Population.Chin Med J (Engl).2017 Aug 5;130(15):1768-1772. doi:10.4103/0366-6999.211538National Center for Advancing Translational Science Genetic and Rare Diseases Information Center.Progressive Bulbar Palsy.USA Social Security Administration Program Operations Manual System.Progressive Bulbar Palsy.Bublitz SK, Weck C, Egger-Rainer A, Lex K, Paal P, Lorenzl S.Palliative Care Challenges of Patients With Progressive Bulbar Palsy: A Retrospective Case Series of 14 Patients.Front Neurol.2021 Sep 24;12:700103. doi:10.3389/fneur.2021.700103Hopwood V, Nanjing DA, Donnellan C.Motor Neurone Disease.Acupuncture in Neurological Conditions.2010:173-183. doi:10.1016/B978-0-7020-3020-8.00011-4
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Zhang HG, Chen L, Tang L, Zhang N, Fan DS.Clinical Features of Isolated Bulbar Palsy of Amyotrophic Lateral Sclerosis in Chinese Population.Chin Med J (Engl).2017 Aug 5;130(15):1768-1772. doi:10.4103/0366-6999.211538National Center for Advancing Translational Science Genetic and Rare Diseases Information Center.Progressive Bulbar Palsy.USA Social Security Administration Program Operations Manual System.Progressive Bulbar Palsy.Bublitz SK, Weck C, Egger-Rainer A, Lex K, Paal P, Lorenzl S.Palliative Care Challenges of Patients With Progressive Bulbar Palsy: A Retrospective Case Series of 14 Patients.Front Neurol.2021 Sep 24;12:700103. doi:10.3389/fneur.2021.700103Hopwood V, Nanjing DA, Donnellan C.Motor Neurone Disease.Acupuncture in Neurological Conditions.2010:173-183. doi:10.1016/B978-0-7020-3020-8.00011-4
Zhang HG, Chen L, Tang L, Zhang N, Fan DS.Clinical Features of Isolated Bulbar Palsy of Amyotrophic Lateral Sclerosis in Chinese Population.Chin Med J (Engl).2017 Aug 5;130(15):1768-1772. doi:10.4103/0366-6999.211538
National Center for Advancing Translational Science Genetic and Rare Diseases Information Center.Progressive Bulbar Palsy.
USA Social Security Administration Program Operations Manual System.Progressive Bulbar Palsy.
Bublitz SK, Weck C, Egger-Rainer A, Lex K, Paal P, Lorenzl S.Palliative Care Challenges of Patients With Progressive Bulbar Palsy: A Retrospective Case Series of 14 Patients.Front Neurol.2021 Sep 24;12:700103. doi:10.3389/fneur.2021.700103
Hopwood V, Nanjing DA, Donnellan C.Motor Neurone Disease.Acupuncture in Neurological Conditions.2010:173-183. doi:10.1016/B978-0-7020-3020-8.00011-4
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