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/315632 overview 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-barre syndrome/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.