Progressive bulbar palsy is a motor neuron disease (MND) that damages the nerve cells in the brain stem that supply the bulbar muscles -- those that control talking, swallowing, and chewing. Other areas of the body may also be affected by this disease.
The onset of this disease generally 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 is usually slow to start, but aggressive. Many times, a person who is diagnosed with this disease will only have a few years to live.
The main symptom of this condition is muscle weakness that causes difficulty chewing, talking, 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 he can not move his tongue, making eating food difficult or impossible. Speaking usually also becomes hard, 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 with no warning, and for no reason. At times, 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 noticed right away.
Aspiration pneumonia is often the cause of death for those who have this aggressive motor neuron disorder. Inability to swallow properly or gagging can result in a person inhaling the food or drink he is consuming. When this occurs, the food or drink goes down into the lungs, increasing the chance of a person getting this pneumonia.
Treatment for progressive bulbar palsy is generally management of symptoms. Certain types of medications may be used to reduce muscle spasms and any pain associated with the degenerating muscles. Physical therapy is often recommended to keep muscles in action. In some cases, throat surgery may help an affected person be able to swallow. A nurse or qualified caregiver may be called in to assist a person with eating, if surgery is not an option or has failed to work. To help with changing emotions and depression that often occurs with progressive bulbar palsy, a patient’s doctor may prescribe an anti-depressant.
