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Guillain–Barré syndrome
By: Dacy Gaston, RN, BSN
What is it?

Guillain-Barre syndrome is a serious disorder that
occurs when the body's defense (immune) system
mistakenly attacks part of the nervous system. This leads
to nerve inflammation that causes muscle weakness and
other symptoms (Grossman & Port, 2014)
Who Discovered it?

Octave Landry is credited with the first reported case of what later came
to be known as GBS. In 1859, Landry described a neurologic condition
characterized by ascending motor paralysis with poor prognosis that he
referred to as "ascending paralysis” AKA “Landry’s paralysis” (Afifi, 1994)
Figure 1. Octave Landry. Adapted from Wikipedia, retrieved from http://en.wikipedia.org/wiki/Jean_Landry_(physician)
Who Discovered it?

Later Guillain Barré syndrome was coined by the French physicians JeanAlexandre Barré, Georges Guillain and Andre Strohl in the early 1900s
following tests on soldiers returning from World War 1. Many cases of an
identical condition had been described over the preceding 80 years but these
neurologists identified one of the characterizing features of the disease – the
increased concentration of protein in the spinal fluid without evidence of
inflammation (Afifi, 1994) No one knows why Strohl wasn’t included in the
naming of the disease….
Figure 2. Guillain, Barre, Strohl. Adapted from http://www.Wikipedia.org
Pathway to disease

Upper Respiratory infection or gastrointestinal infection often precedes the onset
of GBS.

All forms of Guillain–Barré syndrome are autoimmune diseases, due to an immune
response to foreign antigens.

Ascending paralysis ensues as the disease progresses.

The end result of this autoimmune process is an attack on the peripheral nerves
and damage to myelin, the fatty insulating layer of the nerve, and a nerveconduction block leading to muscle paralysis that may be accompanied by sensory
or autonomic disturbances.

Guillain–Barré, unlike disorders such as multiple sclerosis (MS) and Lou Gehrig's
disease (ALS), is a purely peripheral nerve disorder and does not in general cause
nerve damage to the brain or spinal cord (Caple, 2013)

Can mimic these diseases so do a proper patient history and work up.
Signs and Symptoms

The disorder is characterized by symmetrical weakness that usually affects
the lower limbs first, and rapidly progresses in an ascending fashion. Patients
generally notice weakness in their legs.

Numbness and tingling may or may not be present.

As the disease progresses the arms and facial muscles become affected.


Bulbar weakness

Oropharyngeal dysphagia-drooling, difficulty swallowing or difficulty maintaining
an open airway.

Facial weakness

Repertory distress may require ventilation.

Sensory loss, proprioception and areflexia are important factors of GBS.
In severe cases of GBS, loss of autonomic function is common, manifesting as
wide fluctuations in blood pressure, orthostatic hypotension, and sinus
tachycardia among other cardiac arrhythmias (Caple, 2013)
Scary Stuff (Well, kinda scary):

Can happen really fast!!!

Post infection the disease can progress within days to cause severe symptoms.

Sometimes hospitalization is required if the inflammation moves to the nerves
that are in the diaphragm and breathing is being affected.

Don’t worry though TOOOOO much,

Only about 1.65%-1.79% per 100,000 people a year are affected

And only 3% of those percentages is fatal

Recurrence rate is only about 3% (Caple, 2013)
Risk Factors

Men > Women

Infectious agents such as:

Campylobacter jejuni enteritis --- most associated (Koga, 2006)

Epstein-Barr Virus

Cytomegalovirus

Mycoplasma

HIV

Influenza

Systemic illness : i.e. Hodgkin's lymphoma, SLE

Vaccinations
(Caple, 2013)
Interesting Fact:

Following the 1976 H1N1 Vaccine, there was an increase in the
reported incidences of GBS. Studies showed that there was a link,
although small, associated with that particular vaccine and GuillainBarre Syndrome.

In response to the 2009 H1N1 scare, there was obviously a curiosity in
the link and a study was done to show that there was still a small, but
measurable risk, with the pH1N1 vaccine and developing GBS (Wise,
et.al, 2012)

BUT!!!!!--- the benefits far outweigh the risks of not getting the
vaccine, so therefore was deemed not a significant risk factor to not
getting vaccinated.
Diagnostic and Testing

The diagnosis of GBS depends on findings such as rapid development of
muscle paralysis, areflexia, absence of fever, and a likely inciting event.
Cerebrospinal fluid analysis (through a lumbar spinal puncture) and
electrodiagnostic tests of nerves and muscles (such as nerve conduction
studies) are common tests ordered in the diagnosis of GBS
CSF
characteristic findings include albumino-cytological dissociation. As opposed to
infectious causes, this is an elevated protein level (100–1000 mg/dl), without an
accompanying increased cell count (absence of pleocytosis). A sustained increased white
blood cell count may indicate an alternative diagnosis such as infection.
EMG and nerve conduction studies
may show prolonged
distal latencies, conduction slowing, conduction block, and temporal dispersion of
compound action potential in demyelinating cases. F waves and H-reflexes may be
prolonged or absent. Needle EMG is frequently normal in acute cases. Reduced,
neuropathic recruitment in weak muscles can be seen. Fibrillations will be seen on needle
EMG if some axonal injury occurs after three to four weeks. In primary axonal damage,
the findings include reduced amplitude of the action potentials without conduction
slowing (Grossman & Port, 2014)
Treatment

Supportive care is the cornerstone of successful management in the acute
patient. Of greatest concern is respiratory failure due to paralysis of the
diaphragm. Intubation may be needed when evidence of impending failure
of the muscles of breathing is present

Subsequent treatment consists of attempting to reduce the body's attack on
plasmapheresis, filtering antibodies out
of the blood stream, or by administering intravenous
immunoglobulins (IVIg), to neutralize harmful antibodies and
the nervous system, either by
inflammation causing disease. These two treatments are equally effective and
a combination of the two is not significantly better than either alone
(Grossman & Port, 2014)
Patient Teaching Post Infection

Manage psychosocial aspects of GBS (anxiety, education)

Full recovery may take up to two years with strict adherence to rehab
regimen.

Support groups may be beneficial.
(Caple, 2013)
IN CONCLUSION………
A picture is worth a thousand words….
Questions!

1) A 28 year old male comes to you complaining of profound weakness and
numbness in his feet that he said has “seemed to get worse and moving up his
legs” he just got over a really bad stomach flu a few days ago. You may
suspect

A) B12 deficiency

B) Bad sushi from downtown Savannah

C) Guillian Barre symptoms

D) The feeling you get right before you take a patho test.
Questions

2) Guillain Barre symptoms can be life threatening if not treated promptly
and correctly?

A) True

B) False
Questions

3) Guillain Barre Syndrome is a demyelination of the myelin in:

A) The Central Nervous System

B) The Peripheral Nervous System
Questions:

4) Which medications are used to treat Guillain Barre Syndrome? Check all
that apply

A) Doxycycine

B) IvIg

C) Plasmapheresis

D) Supportive therapy
Questions:

5) Who is your favorite South University professor?

A) Dr. Seus

B) Dr. Gupta

C) Dr. Mokoko

D) Dr. House
References:
Afifi, A. (1994) The Landry-Guillain-Barre-Strohl Syndrome 1859 to 1992 a historical perspective. Journal of
Family and Community Medicine, 1(1), 30-34.
Caple, C. (2013). Guillain-Barre Syndrome. Retrieved from www.questhealth.com
Grossman, S., Porth, C. (2014). Disorders of Motor Function: Guillain-Barre Syndrome. Porth’s
Pathophysiology: Concepts of Altered Health States (p. 467). Philadelphia, PA: Wolters Kluwer
Health/Lippincott Williams & Wilkins.
Koga, M., Gilbert, M., Takahashi, M., Li, J., Koike, S., Hirate, K., Nobuhiro, Y. (2006). Comprehensive analysis
of bacterial risk factors for the development of Guillain-Barre syndrome after campylobacter jejuni enteritis.
The Journal of Infectious Diseases, 193, 547-555.
Wise, M., Viray, M., Sejvar, J., Lewis, P., Baughman, A., Connor, W., Giambrone, R., Hale, C., Hogan, B., Meek,
J., Murphree, R., Oh, J., Reingold, A., Tellman, N., Conner, S., Singleton, J., Lu, P., DeStefano, F., Fridkin, S.,
Vellozzi, C., Morgan, O. (2012) Guillain-Barre syndrome during the 2009-2010 H1N1 influenza vaccination
campaign: population based surveillance among 45 million americans. American Journal of Epidemiology,
175(11), 1110-1119.