Guillain-Barré
Syndrome
Heather Hetler
CD 502 Motor Speech Disorders
Spring, 2011
What is Guillain-Barré Syndrome?
• Guillain-Barré Syndrome is an idiopathic, acute, progressive,
inflammatory, peripheral neuropathy, characterized by
progressive muscle weakness over hours to several days.
• May involve degeneration of axons, demyelination, or a
combination of both, though demyelination is most common.
• Considered a group of disorders rather than a single disease.
• An autoimmune disease, involving the immune system
attacking the myelin (in demyelination types of GBS)
• Most important cause of acute flaccid paralysis in the United
States, but still rare, with a prevalence between 1.5 and 3
cases per 100,000 per year in North America.
Causes
• The exact triggers or causes of Guillain-Barré Syndrome (GBS)
are unknown.
• It often follows a minor respiratory or abdominal infection.
• May occasionally follow a vaccination:
• The swine flu vaccine in 1976 is suspected to have caused several
cases.
• According to the CDC, “As of February 25, 2008 … Vaccine
Adverse Event Reporting System (VAERS) has received 26
confirmed case reports of GBS within 6 weeks of receipt of MCV4
Menactra meningococcal vaccination. ” This indicates there may
be a small increased risk, though the data is not conclusive.
• The risk, if in fact present, is very slight, compared to the risk
presented from the diseases for which vaccines are administered.
Symptoms
• Most common type of GBS seems to start with weakness in
legs, gradually ascending to arms and head.
• Weakness is most often bilateral, unlike a stroke.
• Weakness in legs can cause difficulty with walking, general
clumsiness, and difficulty going up and down stairs.
• As the weakness ascends and affects arms and hands,
difficulty is seen in holding up arms for self-care tasks
(brushing teeth, combing hair) and difficulty with fine motor
tasks (writing, buttoning clothing).
Symptoms
• Approximately 40% of those with GBS will have respiratory
system muscles impacted, and one fourth will require
respiratory support.
• Difficulty taking breath or deep breath, difficulty with coughing, feeling
of suffocation.
• Cranial Nerve involvement in one half of those affected.
• Seventh cranial nerve (facial nerve) damage leads to inability to
smile or fully close eyes.
• Ninth (Glossopharyngeal) and Tenth (Vagus) cranial nerve damage
may cause weakness in throat musculature which compromises
airway, causing choking even on saliva.
• Occasionally damage to Twelfth (Hypoglossal) cranial nerve,
resulting in weakness of tongue and affecting speech.
• Rarely, loss of all voluntary muscle movement, resulting in a
“Locked-in” phenomenon.
Sensory Symptoms
• Abnormal sensations (paresthesias) present in 50-70% of
those with GBS.
• Include “pins and needles” sensation, tingling, numbness
• First occur hours to days before muscle weakness.
• Painful muscle cramping in 30% of patients.
• Between shoulder blades, lower back, thighs
• Other sensory symptoms include: feeling of insects crawling
on body, sense of vibration, sense of pressure.
• Loss of sensation, such as ataxia
• May cause abnormal gait due to lack of proprioception
• May be undetectable until neurological exam.
Symptoms
• Loss of reflexes is undetectable to patient, but is notable and
may help to make a diagnosis.
• Deep tendon reflexes (muscle stretch reflexes), commonly elicited by
tapping the tendon just below knee, leading quadriceps muscle to
contract and jerk leg forward.
• More than 80% of patients may present with pain.
• Often unrecognized and undertreated by doctors, who may
believe pain is not part of GBS.
• May predate weakness, and also occurs during recovery and
rehabilitation.
• Pain level in proportion to level of weakness.
Symptoms
• Damage to autonomic nerves, part of peripheral nervous
system that automatically regulates internal organs.
• Increased heart rate most common.
• Difficulty urinating, heart palpitations, constipation, low or high
blood pressure are some possible symptoms.
Diagnosis
• Can be difficult to diagnose early, compromising effective
treatment.
• Antecedent event, such as virus, may be assumed cause of
weakness, particularly in children.
• New weakness after surgery is difficult to note.
• Several other diseases resemble GBS, making diagnosis more
difficult, such as:
• Polio, though nearly eradicated in Western world, can still occur
in those from other countries who have not been vaccinated, or
in older non-vaccinated adults recently exposed to young
vaccinated babies or children.
• West Nile Virus
• When facial paralysis is unilateral, often mistaken for Bell’s Palsy.
Diagnosis, continued
• Several more rare forms of GBS complicate diagnosis as they
follow a different pattern.
• Miller Fisher Syndrome (MFS)
• Sensory GBS: never develop weakness, only sensory symptoms
• Bulbar GBS: begin in muscles around face and throat, causing
difficulty swallowing and slurred speech, usually descends to
involve limbs.
• Axonal GBS: damage to axons in addition to demyelination.
Approximately 10% of all cases. Rapid progression of paralysis,
more severe, and prognosis is poor.
Diagnosis
• Symptom diagnosis: The medical personnel take a case
history, and identify symptoms, and make a diagnosis based
on information.
• Nerve conduction studies: electrical testing of nerve function,
which can confirm diagnosis of GBS and exclude other
disorders of acute, progressive weakness.
• Electrical shocks are administered through the skin to determine nerve
function, by examining time it takes current to go from stimulation to
recording site.
• Cerebral Spinal Fluid Testing (lumbar puncture): increased
protein concentration with normal number of cells.
• If the protein level is above the established threshold, then
investigate other disease or disorder, such as West Nile.
Treatment
• Because the severity of GBS can vary widely, it is often
recommended to hospitalize any patient with suspected or
confirmed GBS to monitor during the acute phase.
• Vital signs (including breathing, heart rhythm, blood pressure,
and swallowing) should be monitored closely.
• Watch for complications, such as additional infections like
pneumonia.
• Individual complications will be treated (such as breathing
difficulties, high blood pressure), but there is no immediate
cure for GBS.
Treatment
• The SLP will be involved when cranial nerves affecting
swallowing and speech are impacted.
• Symptoms of drooling, gagging, and choking should result in
stopping of eating until an assessment can be done.
• A bedside swallow exam may indicate the need for a video
swallow study.
• The monitoring of thirst and hydration is also very important in a
paralyzed patient who cannot self-monitor hydration.
• Rehabilitation should begin as soon as patient is medically
stable, and continue after need for acute care has passed.
• Occupational and Physical Therapy, including range of motion
exercises, splints, positioning
Treatment
• Speech Therapy in Rehabilitation: For patients who have had
swallowing and speech affected, therapy within rehabilitation
will be necessary.
• Those who required a tracheostomy may also need speech therapy in
rehabilitation to regain ability to speak clearly.
• Rehabilitation may need to continue into the home, with
accommodations made to home environment.
References
• Brandt, A.K. (2002). Learning to Walk Again: How Guillain Barre
Taught Me to Walk a Different Path. Lincoln, NE: iUniverse, Inc.
• Davids, H.R., MD (2010, March 29). Guillain-Barre Syndrome.
Retrieved from http://emedicine.medscape.com/article/315632overview
• GBS/CIDP Foundation International (n.d.). Guillain-Barré Syndrome.
Retrieved from http://www.gbs-cidp.org/index.html
• Mayo Clinic Staff (2009, May 30). Guillain-Barre syndrome. Retrieved
from http://www.mayoclinic.com/health/guillain-barresyndrome/DS00413
• National Institute of Neurological Disorders and Stroke (2011, March
23). Guillain-Barré Syndrome Fact Sheet. Retrieved from
http://www.ninds.nih.gov/disorders/gbs/detail_gbs.htm
• Parry, G.J. (2007). Guillain-Barré Syndrome: From Diagnosis to
Recovery. New York, N.Y.: Demos Medical Publishing.