What Is Guillain-Barré syndrome?
Neurologist answers 11 questions on rare disorder in the news right now
The news that Dallas Cowboys center Travis Frederick had been diagnosed with a neurological disorder called Guillain-Barré syndrome shocked fans.
You may be even more surprised to learn that while GBS is rare (incidence is about 2 per 100,000 cases), Cook Children's neurologists see between 6 to 10 cases per year.
1. What is GBS?
It is an auto-immune process in which your immune system starts attacking the myelin which is the protective coating that surrounds your nerves. As a result, the information needing to get down the nerve to communicate with other parts of the body is slowed or unable to occur.
2. What are the causes?
Most common cause is almost always post-infectious, either viral or bacterial. Most common bacterial cause is Campylobacter jejuni which causes a gastrointestinal illness. It is not an infection itself and is not contagious. Symptoms usually start within a few weeks of the illness. Occasionally, it can be associated with vaccinations or post surgically.
3. What are the symptoms?
Symptom onset is usually relatively rapid and can include: progressive, ascending weakness (starts in feet and maybe hands and spreads gradually up the legs, arms and trunk), abnormal sensations such as pain, tingling, numbness and pins and needles, and autonomic instability (abnormal heart rate, blood pressure, temperature stability, breathing rate)
In more severe cases, difficulty breathing, facial weakness, trouble swallowing, and bowel/bladder dysfunction.
Peak of symptoms is usually within 2-3 weeks after onset.
4. What is the treatment?
IVIG is the treatment of choice and in more severe cases may require plasmapheresis. The sooner treatment is initiated the better the outcome. We prefer for treatment to start within 7-10 days of onset of symptoms.
Definition of IVIG: Immunoglobulins are special proteins in the blood. They fight infections. Some white blood cells make them. They are also known as antibodies. They are part of the immune system. With immunoglobulin therapy (IVIg), these proteins are donated from a healthy person. They are then given to you through an IV.
5. Is there an age range when you are more susceptible to it?
Peak age for onset of GBS is 38-40 years of age, but any age may be affected.
6. Does it impact children differently than adults?
The type of symptoms and response to treat is usually similar in children and adults
7. When should you see a doctor?
Any quick onset of abnormal sensations and/or weakness that is worsening needs to be evaluated. If the weakness is progressing quickly, may need to be evaluated in emergency department. Many times, people have difficulty with ambulating.
8. Are there different types of GBS?
There are axonal variants such as AMAN (acute motor axonal neuropathy) and AMSAN (acute motor and sensory axonal neuropathy) in which the immune system is attacking the membrane of the nerve itself not the protective coating. With AMAN, you do not usually have sensory disturbances associated with the disease. AMSAN can look very similar to GBS and there has been some debate in the past if they are the same or different diseases. AMSAN usually has a more rapid progression of symptoms over days and can take longer to recover.
Miller Fisher syndrome is onset of weakness that usually starts in the face and includes difficulty with moving the eye muscles leading to droopy eyelids and double vision, ataxia which is unsteadiness and incoordination, and decrease in your reflexes. Weakness can spread to rest of body in a more of a descending pattern. This should not be confused with botulism which is also a descending weakness but usually also affects the pupils.
There is also tick paralysis which can look like GBS so need to also look for tics on the body if have similar symptoms.
9. How rare is it to get it later in like like Travis Frederick?
It is common to get it in the adult age. Peak age for onset of GBS is 38-40 years of age.
10. Once you’ve had it, can it recur? Is it something that lasts forever?
There is a chronic form of GBS called CIDP or chronic inflammatory demyelinating polyradiculoneuropathy in which progression of symptoms continues at least 8 weeks and can be more difficult to treat. Most of the time, GBS is an illness you only develop once. If symptoms recur, then again, it may be that you have the chronic form that will need long term immunosuppressive or immunomodulating medications.
Most people have full or near full recovery of symptoms but this can take months or for more severe cases even years.
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When it comes to your child's brain, spinal cord and nervous system, you want the very best team of neuroscience specialists available.
Pediatric neurology deals with diseases, disorders and injuries that can affect a child's brain, spinal cord, and all associated blood vessels, muscles and nerves. If your child has a problem involving the nervous system, Cook Children's pediatric neurologists have the special training and experience necessary to diagnose and treat your child.