What is progressive bulbar palsy?

Progressive bulbar palsy is a disorder that damages the bulbar muscles in the cerebral cortex, spinal cord, and brainstem of an affected person. It is classified as a motor neuron disease (MND). As it generally affects the areas of the brain that control speaking, swallowing, and chewing, these functions are usually affected by the condition. Other areas of the body can also be affected by this disease.

The onset of this disease usually occurs in people between 50 and 70 years of age. When it develops in children, it is often referred to as infantile progressive bulbar palsy. The disorder usually begins slowly but is aggressive. Many times, a person diagnosed with this disease will only have a few years to live.

The main symptom of progressive bulbar palsy is muscle weakness that causes difficulty chewing, speaking, and swallowing. Weak jaws, throats, and facial muscles are also frequently reported, along with drooling and choking. A person with this disease may find that they cannot move their tongue, which makes eating food difficult or impossible. Speaking usually also 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 happen 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 is not immediately noticeable.

Aspiration pneumonia is often the cause of death for those who have this aggressive motor neuron disorder. The inability to swallow properly or nausea can cause a person to inhale the food or drink that he is consuming. When this happens, food or drink travels down into the lungs, increasing a person's chance of getting this pneumonia.

Treatment for progressive bulbar palsy is generally symptom management. Certain types of medication can be used to reduce muscle spasms and any pain associated with muscle degeneration. Physical therapy is often recommended to keep the muscles in action. In some cases, throat surgery can help an affected person to be able to swallow. A nurse or skilled caregiver 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 occur with progressive bulbar palsy, the patient's doctor may prescribe an antidepressant.

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