Progressive bulbar palsy is a motor neuron disease that affects the nerve cells in the brainstem that control speaking, swallowing, and chewing. It usually affects people between 50 and 70 years old and can cause muscle weakness, difficulty speaking and swallowing, and emotional lability. Treatment is usually focused on managing symptoms.
Progressive bulbar palsy is a motor neuron disease (MND) that damages the nerve cells in the brainstem that power the bulbar muscles, those that control speaking, swallowing, and chewing. Other areas of the body can also be affected by this disease.
The onset of this disease usually occurs in people between the ages of 50 and 70. When it develops in children, it is often referred to as infantile progressive bulbar palsy. The disorder is usually slow to start but aggressive. Many times, a person diagnosed with this disease will only have a few years to live.
The main symptom of this condition is muscle weakness which causes difficulty chewing, speaking and swallowing. Weak jaws, throats, and facial muscles are also often reported, along with drooling and choking. A person with this disease may find that they cannot move their tongue, making it difficult or impossible to eat food. Speaking also usually becomes difficult, and eventually a person with this may not be able to speak at all.
Other symptoms, such as uncontrollable crying or laughing, may also occur. These are known as emotional lability and often occur without warning and for no reason. Sometimes, progressive bulbar palsy can even affect a person’s arms or legs, making them weak. Sometimes, this loss of strength in the extremities is so subtle that it’s not immediately noticeable.
Aspiration pneumonia is often the cause of death for those with this aggressive motor neuron disorder. The inability to swallow properly or vomiting can lead a person to inhale the food or drink they are consuming. When this occurs, food or drink goes down into the lungs, increasing the chance that a person will get this pneumonia.
Treatment for progressive bulbar palsy is usually symptom management. Certain types of medications can be used to reduce muscle spasms and any pain associated with muscle wasting. Physical therapy is often recommended to keep the muscles moving. In some cases, throat surgery can help an affected person swallow. A trained nurse or assistant may be called in to help a person eat if surgery is not an option or has not worked. To help change the emotions and depression that often occurs with progressive bulbar palsy, a patient’s doctor may prescribe an antidepressant.
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