A Case of Guillain-Barre Syndrome

in a Primary Care Setting

Sherly Sebastian, DNP, RN, NP-C

ABSTRACT
Guillain-Barre Syndrome (GBS) is an immune-mediated peripheral neuropathy
characterized by rapidly progressive symmetrical ascending weakness and sensoiy
loss. The disease can progress rapidly and be fatal i f diagnosis and treatment interven-
tions are delayed. I n most patients, the neuropathic symptoms are preceded by an
antecedent infection. The patient may present w i t h initial symptoms o f upper respi-
ratory tract infection or gastroenteritis. This article presents a case report o f GBS, fol-
lowed by a detailed discussion o f the pathophysiology, diagnostic studies, differential
diagnosis, types, prognosis, and management o f patients w i t h this condition.

Keywords: ascending weakness, immune response, peripheral neuropathy,

respiratory failure, sensory loss
2012 American College of Nurse Practitioners

G uillain-Barre Syndrome (GBS) is an i m m u n e -

mediated peripheral neuropathy characterized
by rapidly progressive symmetrical ascending
weakness and sensory loss. The disease is named after the
physicians Guillain, Barre, and Strohl, w h o described the
clinical presentation in 2 French soldiers during the First
World War about 100 years ago.' According to the
National Institute o f Neurological Disorders and Stroke
figures,' the annual incidence rate for GBS is about 1-2
per population o f 100,000. I n the acute phase o f GBS,
approximately 3% o f patients may die from acute c o m p l i -
cations and up to 20% have residual, permanent, severe
disabilit)' w i t h ambulation deficits or require ventilator
assistance up to 12 months later,-'
The annual financial burden from GBS is estimated at
$1.7 bilhon, including 10.2 bilhon (14%) in direct med-
ical costs and $1.5 billion (86%) in indirect costs from
lost productivity or premature death.** Even though inci-
dence is low, the economic cost o f GBS is substantial as a
result o f high mortahty and morbidity.
CASE REPORT
This patient is a 36-year-old Hispanic woman w h o pre-
sented to the outpatient clinic w i t h complaints o f numb-
ness, tingling, and weakness in her legs. The patient was
initially seen in the clinic 3 weeks ago w i t h upper respi-
ratory tract infection and was treated w i t h a course o f
amoxicillin. Her upper respiratory symptoms improved,
but a week later she noticed numbness and tingling in
her feet, progressively ascending to her legs and thighs.
She was evaluated at the local hospital, and her diagnostic
work-up included blood cultures and spinal tap.The
emergency r o o m (ER) physician consulted the neurolo-
gist, but the patient left E R against medical advice.
^ T h e numbness became progressively worse, and she
started having bilateral lower extremity weakness and gait
issues. She fell 2 days ago, and her family forced her to
seek medical help. Her past medical history was unre-
markable except for the upper respiratory infection
( U R I ) 3 weeks ago. The patient's review o f systems was
negative for shortness o f breath, fever, chilis, cough, and
swallowing difficulties. H e r vital signs were temperature
3 7 C , pulse 112/minute, blood pressure 108/62, respira-
tion 28/minute, weight 162 lbs, height 5'2".
O n neurological examination, the patient was alert and
oriented X 3, w i t h intact speech, comprehension, and mem-
017; pupils equal round reactive to light. Dysmetria was
noted for finger-to-nose and rapid alternating movements

www.npjournal.org The Journal for Nurse Practitioners - J N P 643

Table 1. Guillain-Barre Syndrome Variants
Acute Inflammatoty Demyelinating Polyneuropathy (AIDP)^'
Most prevalent in United States and Europe
Immune response at myelin sheath and Schwann cells
Cranial and sensory nerves more affected than motor
nerves
Acute Motor Axonal Neuropathy (AMAN)
More prevalent in pediatric group
Immune response is against motor axon membranes
Motor involvement without sensory findings
Acute Motor and Sensor Axonal Neuropathy (AMSAN)
More rapid progression and paralysis with sensory and
motor deficits
Axonal degeneration of ventral and dorsal nerve roots
Prolonged recovery with painful sensory component
Miller-Fisher Syndrome (MFS)
Rare subtype, classic findings of areflexia, ataxia, and
ophthalmoplegia
Acute Panautonomic Neuropathy
Very rare subtype affecting both sympathetic and
parasympathetic systems
Recovery is slow and incomplete, with high mortality
and morbidity
bilaterally. Her cranial nerve exam was intact. Her motor
strength in triceps, biceps, and supinator were 5/5; deltoids
and pronator were 4/5; wrist extensors and flexors were
4/5 bilaterally; hip flexors, quadriceps, and hamstrings
were 4/5 bilaterally; dorsiflexors and plantar flexors were
3/5 bilaterally. Her sensation was intact on upper extremities
but diminished to touch and pin prick on lower extremities
and abdomen. Her gait was ataxic, deep tendon reflex
( D T R ) 1 + and symmetrical on deltoid, biceps, brachio-
radialis and triceps; D T R was absent on knees and ankles.
The patient's laboratory studies did not reveal any
infectious process. H e r cerebrospinal fluid (CSF) studies
from the hospital revealed elevated protein count o f
200 m g / d L , w i t h normal cell count, w h i c h confirmed
the diagnosis o f GBS. The disease can progress rapidly
and can be potentially fatal i f treatment interventions are
delayed. The patient was transported expeditiously to the
nearest hospital for admission to the intensive care unit.
DIAGNOSIS
GBS is a heterogeneous syndrome w i t h several variant
forms affecting the peripheral nervous system as a result
o f an immune-mediated disturbance involving the
644 The Journal for Nurse Practitioners - JNP
Table 2. Clinical Manifestations^"
A. Clinical symptoms definitive of GBS
Bilateral symmetrical ascending motor weakness,
areflexia
B. Clinical symptoms supportive of GBS
Ataxia, cranial nerve palsies
Respiratory muscle weakness and paralysis
Numbness/paresthesia on extremities
Decreased proprioception
Pain in the lower extremities and back, fatigue
Bilateral facial weakness, difficulty with eye
movements
Chewing and swallowing difficulties
Autonomic dysfunction
C. Clinical symptoms not supportive of GBS
Unilateral weakness, hyperreflexia
Sudden onset of weakness
Changes in level of consciousness
Asymmetry of weakness and paresthesia
Severe paresthesia without motor weakness
Severe respiratory symptoms with limited motor
weakness
Bladder or bowel dysfunction at the onset of
symptoms
peripheral myelin sheath. The commonly recognized
variants are summarized i n Table 1.
The patient in the case study provides many o f the
classic characteristics o f GBS, such as progressive
development o f ascending symmetrical paresthesia,
pain, bilateral m o t o r weakness, areflexia, and ataxia.
Accurate diagnosis requires clues from the clinical his-
tory, such as onset, severity', and rate o f progression o f
the symptoms over hours to days. A careful n e u r o l o g i -
cal exammation m c l u d i n g the m o t o r strength, cranial
nerves, and reflexes is essential. Clinical manifestations
are summarized i n Table 2.
Antecedent Infections
The history o f a prodromal respiratory infection should
raise suspicion for GBS, because in majority o f GBS
patients, neuropathic symptoms are preceded w i t h a U R l
or enteric infection. History o f cough, fever, sore throat,
diarrhea, or any other types o f infections 2-3 weeks
before the onset o f weakness should alert the clinician to
suspect GBS. Campylobacter jejuni, a bacteria commonly
associated w i t h gastroenteritis, have been identified as the
most frequent antecedent pathogen o f GBS.'
Volume 8, Issue 8, September 2012
Vaccinations
The evolution o f GBS after vaccination has been studied
extensively, as concerns about vaccine-induced GBS were
initially raised after the 1976-77 influenza vaccination
period.The landmark study done by Lasky et al '
reported marginally increased risk o f GBS after influenza
vaccination during the first 6 weeks o f immunization.
Subsequently, researchers investigated the relationship
between GBS and the influenza vaccinations and
reported that low relative risks were not statistically sig-
nificant.'"Extensive research is reported after the H l N l
mass vaccination i n 2009 and found no evidence o f an
increased risk o f GBS after the seasonal influenza vaccine
or the H l N l vaccination."
Diagnostic Studies
CSF analysis w i t h evidence o f high protein and normal
cell count support the diagnosis o f GBS. The value o f
lumbar puncture is limited i n the early phase o f the dis-
ease. CSF may remain normal in up to 50% o f patients
early i n the disease, but elevated protein w i l l be present
i n more than 90% o f the patients by the time they reach
clinical nadir.'* However, increased CSF ceD count steers
cUnician to consider other diagnoses and investigate pos-
sibility o f infectious process, such as leptomeningeal
malignancy. West N i l e virus infection, HIV, or Lyme dis-
ease.'- I f the CSF results are normal, repeat testing is not
done typically.
Electrodiagnostic testing is done frequently to iden-
tify the acute motor weakness caused by a peripheral
neuropathy. T h e test is helpful i n confirming the diagno-
sis and differentiating the variants o f GBS. However,
early in the disease process, the testing may be normal.
The features o f deniyelination i n GBS include slow con-
duction, temporal dispersion, and prolonged or absent
F-responses.^ Abnormal median and ulnar sensory
potentials w i t h spared sural potentials are seen i n the
initial stages o f GBS.''The nerve conduction study is
considered a useful confirmatory test, w h i c h typically
demonstrates the finding o f reduced muscle action
potentials in chnically weak m u s c l e s . M a g n e t i c reso-
nance imaging ( M R I ) is typically performed to rule out
infiltrative or structural causes o f weakness. Moreover,
M R I may reveal enhancement o f the affected nerve
roots supportive o f GBS diagnosis. The hmitations o f
these tests i n the early phase o f the disease, combined
w i t h the urgency for early treatment, require clinicians
www.npjournal.org
Table 3. Differential Diagnoses^^'^"
Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
S y m p t o m s similar t o GBS but progressing over a longer
period ( > 8 weeks)
Myasthenia Gravis (MG)
Weakness in t h e v o l u n t a r y muscles c o n t r o l l i n g t h e
eyelids, face, s w a l l o w i n g
Weakness and fatigue p r o n o u n c e d w i t h effort and
relieved w i t h rest
Diplopia and/or ptosis, n o r m a l sensation and reflexes
Anterior Horn Cell Abnormalities
S o m e similar features, but weakness pattern is different
Clinical signs of infection, i n c l u d i n g high cell count in
cerebrospinal fluid
Spinal Cord Disorders
Radiculopathy, unilateral m o t o r and sensory deficits
Hyperreflexia, sharp sensory levels
Pain aggravated w i t h activities
Intracranial Abnormalities
Change In level o f consciousness, severe headaches
Unilateral m o t o r and sensory deficits
to make the diagnosis on clinical grounds and promptly
refer to hospital/specialist care.*^''-*
Differential Diagnosis
Ascending symmetrical weakness progressing over hours
to days is the cardinal symptom o f GBS. I f the symptoms
are prolonged over 8 weeks, other diagnoses should be
considered. I f the weakness is asymmetric, clinicians
should consider a spinal or intracranial diagnosis instead
o f GBS. I f there is weakness w i t h intact reflexes, another
diagnosis should be investigated. The predominant fea-
tures o f Miller Fisher Syndrome, such as ophthalmo-
plegia, areflexia, and ataxia, often mistakenly suggest
brain stem infarction. A wide range o f neurological disor-
ders may mimic the symptoms o f GBS; most comnion
differentials are hsted i n Table 3.
PATHOPHYSIOLOGY
The pathogenesis o f GBS has been widely studied but is
still not completely understood.The proposed mecha-
nism involves an antecedent infection leading to an auto-
immune response reacting w i t h peripheral nerve
components (Figure 1). Most o f the pathogens gains
entry to the body through mucosal or gut epithehum
and induce antibody production against specific ganglio-
The Journal for Nurse Practitioners - JNP 645
JNP
r
Figure 1. Guillain-Barre Syndrome Pathogenesis.

Pathogenesis and recovery Disease-modifying factors

Campylobacterjejuni infection Bacterial factors

LOS biosynthesis cluster
Ganglioside mimicry of LOS

Tcell
Immune response to LOS Host factors
Genetic polymorphisms
Immune status

Antigen
presenting
cell

Plasma cell
Cross-reactive antibodies
to nerve gangliosides

^ k h

Macrophage

Extent of nerve damage

Ganglioside distribution in nerves
Nerve dysfunction
Specificity/affinity antibodies
Complement activation

Complement
Conduction dysfunction/block

Clinical prognostic factors

-Age
I Severity at onset
X ' Diarrhoea

Outcome

van Doom PA, Ruts L, Jacobs BC. Clinical features, patfiogenesis, and treatment of Guillain-Barre Syndrome. Lancet Neurol.
2008;7:939-950. Reprinted with permission from Elsevier.

646 The Journal for Nurse Practitioners - JNP Volume 8, Issue 8, September 2012
sides i n myelin/' The immune response depends on bac-
terial factors, such as the specificity o f lipo-ohgosaccha-
ride (LOS), and on host factors, such as genetic
polymorphism and immune status.'"The presence o f
antibodies leads to activation o f T cells and complements,
leading to a cascade o f inflammation and demyelination.
The demyelination decreases the velocity o f nerve con-
duction and slows the impulse transmission along the
axons. The extent o f nerve damage varies, w i t h more
damage seen in the intensely myelinated peripheral
nerves, causing motor and sensory weakness.
The self-limiting nature o f the disease process
should be taken into account, symptoms improve once
the autoimmune inflammatory process is terminated,
and the Schwann cells reverse the process to rebuild the
myelination o f the nerves. I n severe cases, the inflamma-
tory process can lead to axonal disruption and
permanent damage.
SUPPORTIVE CARE
Prevention o f life-threatening complications remains the
cornerstone o f supportive care. Progressive demyehnation
o f the phrenic nerve, innervating the diaphragm, may lead
to acute respiratory muscle paralysis. Early detection o f res-
piratory failure and anticipation o f mechanical ventilation
are crucial to avoid emergency intubation and cardiopul-
monary arrest. Life-threatening episodes o f hemodynamic
instability related to autonomic dysfunction may occur in
GBS patients (Table 4).They should be admitted to the
hospital for close monitoring o f respiratory status, hemo-
dynamic instability, and autonomic dysfunction.
IMMUNOTHERAPY
Immunotherapy should be initiated as soon as possible
w i t h high dose intravenous immunoglobuhn (IVIg) or
plasma exchange (PE).The mechanism o f action o f I V I g
involves modulating the humoral response by suppressing
autoantibody production.^ I V I g also blocks the binding
o f receptors on macrophages, suppressing the various
inflammatory mediators. The typical dose o f I V I g is
0.4g/kg per day for 5 days, started as a lower dose and
increased to the m a x i m u m dosage based on patient toler-
ance. The goal o f PE is to remove circulating i m m u n e -
reactive antibodies, complements, and biological response
modifiers.
The treatment regimen depends on the disease sever-
ity; typically, PE is given 5 times i n 2 weeks, for a total
www.npjournal.org
Table 4. Autonomic Dysfunction^'^^
Tachycardia, bradycardia
Hypotension, hypertension
Facial flushing, anhidrosis, hyperhidrosis
Constipation, paralytic ileus
exchange o f about 5 plasma volumes. The Cochrane
Review'^ demonstrated that I V I g and PE are beneficial
for accelerating the recovery o f GBS, i f given during the
first 4 weeks o f the disease, w i t h most benefit seen i f
given w i t h i n the first 2 weeks o f symptoms.
CORTICOSTEROIDS
The role o f steroids i n GBS treatment has been widely
studied, and researchers concluded that oral corticos-
teroids were not beneficial in GBS treatment and did not
recommend it as the first-line therapy."'This finding is in
contrast to the standard treatment for other demyelinat-
ing diseases for which favorable response is noted w i t h
steroid therapy. I n 1. o f the studies, the use o f intravenous
corticosteroids in combination w i t h I V I g demonstrated a
short-term improvement i n clinical symptoms when
compared to I V I g treatment a l o n e . L o n g - t e r m use o f
corticosteroids causes numerous side effects and may
inhibit macrophage repair process, but short-term
treatment did not result i n serious side effects.'^
PAIN MANAGEMENT
Neuropathic pain occurs i n large number o f patients
w i t h GBS and often requires recognition and treatment.
Nonsteroidal anti-inflammatory drugs (NSAIDs) may be
tried initially, but they often do not provide adequate
pain relief, as the origin o f pain is usually multifactorial.
Pain in the acute phase might be nociceptive related to
inflammation, whereas later in the course, pain may be
related to degeneration o f sensory nerve fibers.'^ Early
recognition and treatment w i t h gabapentin or carba-
mazepine are suggested to be effective i n treating neuro-
pathic pain. Narcotic analgesics should be used w i t h
caution as they precipitate ileus in the setting o f
autonomic dysfunction.
PROGNOSIS
The factors indicating poor prognosis include older age,
infection, prolonged hospital stay, need for mechanical
ventilation and intensive care, and poor upper extremity
The Journal for Nurse Practitioners - J N P 647
Table 5. Complications^'
Respiratory failure, cardiac arrhythmias
Swallowing difficulties, aspiration pneumonia
Deep vein thrombosis, foot drop and joint contractures
Bladder and bowel dysfunction, paralytic ileus
weakness." G B S patients may also exhibit pain, fatigabil-
ity', and functional impairments and may have permanent
neurologic impairments, such as muscle wasting, ataxia,
foot drop, and dysesthesia."^ R e c o v e r y from GBS varies;
most patients recover within 6-12 months, but some may
take longer. T h r o m b o e m b o l i c complications such as deep
vein thrombosis and pulmonary embolus may be pre-
vented with use o f fractionated or unfractionated heparin
and compression stockings.** Early initiation o f an indi-
vidualized program for muscle strengthening and physical
therapy is essential to prevent complications (Table 5).
EDUCATION AND COUNSELING
Clearly, GBS management includes applying an improved
understanding of the pathophysiology to individual
patients and the effect that it has on the vital organs and
tissues. Most patients develop secondary complications,
and tailoring o f supportive care and education should be
based on their unique needs. T h e care of a G B S patient is
challenging for the health care team and the caregivers
because some symptoms can be devastating. G B S is a life
event with a potentially long-lasting influence on physi-
cal and psychosocial well-being, transforming a healthy,
independent person into a critically ill and physically
helpless p e r s o n . ' ' T h e acute progression of motor weak-
ness and fatigue makes a profound effect on the patient's
quahty of hfe.
Several neuromuscular disease organizations provide
resources to assist w i t h community support networks.
T h e Guillain-Barre S y n d r o m e / C h r o n i c Inflammatory
Demyelinating Polyneuropathy Foundation
(http://www.gbs-cidp.org) is an excellent place to
get support, find up-to-date information, and seek
opportunities to network.
CONCLUSION
Even though significant advances have been made
regarding the i m m u n o l o g y and pathophysiology,' o f GBS,
It is still continuing as a challenging neurological diagno-
sis associated with high morbiditx' and mortality. GBS
648 The Journal for Nurse Practitioners - J N P
should be considered in the differential diagnosis for
patients presenting to primary care setting with lower
extremity numbness with antecedent history of upper
respiratory infection or gastroenteritis.The long-term
prognosis is dependent on early diagnosis and treatment
and knowledge o f prognostic factors can substantially
improve patient care. Biti
1. Winer JB. Guillain Barre syndrome. Mol Path. 2001;54(6l:381-385.
2. National Institute of Neurological Disorders and Stroke. Guillain-Barre
syndrome fact sheet. http://wvtfW.ninds.nih.gov/disorders/gbs/detail_gbs.htm.
Accessed July 16, 2012.
3. Khan F, Amatya B, Ng L. Use of the international classification of
functioning, disability and health to describe patient-reported disability: a
comparison of Guillain Barre syndrome with multiple sclerosis in a
community cohort. J Rehabil Med. 2010;42(B):708-714.
4. Frenzen PD. Economic cost of Guillain-Barre syndrome in the United States.
Neurology. 2008;71{l):21-27.
5. Newswanger DL, Warren CR. Guillain-Barre syndrome. Am Fam Physician.
2004;69(1):2405-2410.
6. Pithadia A, Kakadia N. Guillain-Barre syndrome. Pharmacol Rep.
2010;62(l):220-232.
7. Davids HR, Oleszek JL, Cha-Kim A. Guillain-Barre syndrome.
http://emedicine.medscape.com/article/315632. Accessed August 9, 2011.
8. Burns TM. Guillain-Barre Syndrome. Sem Neurol. 2008;28(2):1S2-167.
9. Lasky T, Terracciano GJ, Magder L, et al. The Guillain -Barre syndrome and
the 1992-1993 and 1993-1994 influenza vaccines. N Engl J Med.
1998;339(25):1797-1802.
10. Kuitwaard K, Bos-Eyssen ME, Blomkwist-Markens PH, van Doom PA.
Recurrences, vaccinations and long-term symptoms in GBS and CIDP.
J Peripher Nerv Syst. 2009;14(4):3ia-315.
11. Lehmann HC, Hartung HP Kieseier BC, Hughes RA. Guillain-Barre syndrome
after exposure to influenza virus. Lancet Infect Dis. 2010:10(9):643-65.
12. Van Doom PA, Ruts L, Jacobs BC. Clinical features, pathogenesis, and
treatment of Guillain-Barre syndrome. Lancet Neurol. 2008;7(10):939-950.
13. Winer JB. Guillain-Barre syndrome. Br Med J. 2008;337:227-231.
14. Spalice A, Parisi P Papetti L, et al. Clinical and pharmacological aspects of
inflammatory demyelinating diseases in childhood: an update. Curr
NeuropharmacoL 2010;8(2):135-148.
15. Hughes RA, Raphael JC, Swan AV, van Doom PA. Intravenous
immunoglobulin for Guillain-Barre syndrome. Cochrane Database Syst Rev.
2006;1:CD002063.
16. Bromberg MB, Carter O. Corticosteroid use in the treatment of
neuromuscular disorders: empirical and evidence-based data. Muscle Nerve.
2004;30:20-37.
17. Khan F, Pallant JF Ng L, Bhasker A. Factors associated with long-term
functional outcomes and psychological sequelae in Guillain-Barre
syndrome. J Neurol. 2010;257(12):2024-203.
18. Rudolph T, Larsen JP Farbu E. The long-term functional status in patients
with Guillain-Barre syndrome. Europ J Neurol. 2008;15(12):1332-1337.
19. Bernsen RA, de Jager AE, van der Meche FG, Suurmeijer TP How Guillain-
Barre patients experience their functioning after 1 year. ,4cta Neurol Scand.
2005;112(l):51-56.
Sheiiy Sebastian, DNP, R.N, NP-C, is a luirsc practitioner in
the neurosurgery department at Baylor College of Medicine in
Houston, TX, and can be reached at sherlysebast-
ian@sbcglobal.net. In compliance with national ethical guide-
lines, the author reports no relationships with business or
industry that would pose a conflict of interest.
1565-4156/121/S see front matter
2012 American College of Nurse Practitioners
http://dx.doi.Org/10.1016/j.nurpra.2012.04.015
Volume 8, Issue 8, September 2012