Causes Of Progressive Bulbar Palsy
Progressive bulbar palsy is a rare disease affecting motor neurons in the brainstem or medulla. This part of the brain and nerves are responsible for the most basic functions of the human body including swallowing, chewing, and speaking. A person with progressive bulbar palsy lacks strength in the jaw, tongue, and facial muscles. Swallowing food becomes challenging, and they lose the ability to speak. Other symptoms are a weakness in the limbs and uncontrollable incidents of laughing or crying. Any affliction that interferes with the cranial nerve nuclei can cause this disease. It is considered a type of amyotrophic lateral sclerosis (ALS), and there is no cure. Life expectancy is about two years. Get to know the common causes of progressive bulbar palsy now.
Toxins
Botulinum toxin, a product of the bacterium Clostridium botulinum (C. botulinum), causes progressive bulbar palsy. C. botulinum is commonly found in the ground as spores, which can live in a hibernation-type mode through adverse conditions, coming 'back to life' when conditions are favorable. Humans contract the bacteria by eating improperly cooked or cleaned food. C. botulinum can also be contracted through an open wound. This bacterium releases botulinum toxin, a neurotoxin, that attacks the central nervous system. Other toxins that can cause progressive bulbar include venom from the bark scorpion and some snake venoms.
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Guillain-Barre Syndrome
Guillain-Barre syndrome is a disease in which the immune system attacks the nerves, and it usually starts with tingling or weakness in the feet. Other symptoms include unbalanced gait, fast heart rate, shortness of breath, bladder or bowel incontinence, and difficulty in moving facial muscles. Though the exact cause of Guillain-Barre syndrome is unknown, it usually presents after a digestive or respiratory infection. The protective covering of the nerve cells, or myelin, is damaged to the point where nerve cells cannot communicate. Though it appears to happen in short-lived spurts, paralysis can happen to the entire body. Progressive bulbar palsy has been identified as a variation of Guillain-Barre syndrome.
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Metastasis To The Brain
Metastasis to the brain is the most common cause of brain cancer. This happens when cancer from another part of the body spreads into the brain. The brain tumor is then considered secondary cancer. Most often, cancers from the lungs, kidneys, breast, and melanomas metastasize to the brain using the blood for transportation. This can happen alongside primary cancer or years after the primary cancer has been eradicated. The cancer cells proliferate into a tumor and cause malfunctions in the brain. Only five percent of metastases happen in the brainstem, making this one of the least common ways to develop progressive bulbar palsy.
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Glioma
Glial cells are located throughout the brain along with neurons. They are not directly involved in brain cell communication, but they do play a large part in supporting that communication. They help protect neurons so neural communication can take place. A glioma is a tumor that develops in the glial cells of the brain and spinal cord. This is the most common type of primary brain cancer, and like secondary cancers, these tumors upset normal brain function. Location of the tumor determines what type of treatment can be used, what symptoms to expect, and life expectancy. Tumors on the brainstem usually cannot be operated on because of their proximity to the spinal cord and can cause the symptoms of progressive bulbar palsy.
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Genetic Link
There are few study findings regarding a genetic link as the cause of progressive bulbar palsy. One study found the development of this disease was consistent with a mutation to the SOD1 gene located on chromosome 21. SOD1 produces an enzyme responsible for attaching copper and zinc and eliminating free radicals in the body. Patients with this mutation were more likely to develop amyotrophic lateral sclerosis as well. There is a likelihood the mutations happen in an autosomal recessive pattern. It is not on one of the sex chromosomes and must have two copies to show a mutation.