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A Case of Guillain-Barre Syndrome

in a Primary Care Setting

Sherly Sebastian, DNP, RN, NP-C

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
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
clinical presentation in 2 French soldiers during the First her feet, progressively ascending to her legs and thighs.
World War about 100 years ago.' According to the She was evaluated at the local hospital, and her diagnostic
National Institute o f Neurological Disorders and Stroke work-up included blood cultures and spinal tap.The
figures,' the annual incidence rate for GBS is about 1-2 emergency r o o m (ER) physician consulted the neurolo-
per population o f 100,000. I n the acute phase o f GBS, gist, but the patient left E R against medical advice.
approximately 3% o f patients may die from acute c o m p l i - ^ T h e numbness became progressively worse, and she
cations and up to 20% have residual, permanent, severe started having bilateral lower extremity weakness and gait
disabilit)' w i t h ambulation deficits or require ventilator issues. She fell 2 days ago, and her family forced her to
assistance up to 12 months later,-' seek medical help. Her past medical history was unre-
The annual financial burden from GBS is estimated at markable except for the upper respiratory infection
$1.7 bilhon, including 10.2 bilhon (14%) in direct med- ( U R I ) 3 weeks ago. The patient's review o f systems was
ical costs and $1.5 billion (86%) in indirect costs from negative for shortness o f breath, fever, chilis, cough, and
lost productivity or premature death.** Even though inci- swallowing difficulties. H e r vital signs were temperature
dence is low, the economic cost o f GBS is substantial as a 3 7 C , pulse 112/minute, blood pressure 108/62, respira-
result o f high mortahty and morbidity. tion 28/minute, weight 162 lbs, height 5'2".
O n neurological examination, the patient was alert and
CASE REPORT oriented X 3, w i t h intact speech, comprehension, and mem-
This patient is a 36-year-old Hispanic woman w h o pre- 017; pupils equal round reactive to light. Dysmetria was
sented to the outpatient clinic w i t h complaints o f numb- noted for finger-to-nose and rapid alternating movements The Journal for Nurse Practitioners - J N P 643

Table 1. Guillain-Barre Syndrome Variants Table 2. Clinical Manifestations^"
Acute Inflammatoty Demyelinating Polyneuropathy (AIDP)^' A. Clinical symptoms definitive of GBS
Most prevalent in United States and Europe Bilateral symmetrical ascending motor weakness,
Immune response at myelin sheath and Schwann cells areflexia
Cranial and sensory nerves more affected than motor
B. Clinical symptoms supportive of GBS
Ataxia, cranial nerve palsies
Acute Motor Axonal Neuropathy (AMAN) Respiratory muscle weakness and paralysis
More prevalent in pediatric group Numbness/paresthesia on extremities
Decreased proprioception
Immune response is against motor axon membranes
Pain in the lower extremities and back, fatigue
Motor involvement without sensory findings Bilateral facial weakness, difficulty with eye
Acute Motor and Sensor Axonal Neuropathy (AMSAN) movements
More rapid progression and paralysis with sensory and Chewing and swallowing difficulties
motor deficits Autonomic dysfunction
Axonal degeneration of ventral and dorsal nerve roots C. Clinical symptoms not supportive of GBS
Prolonged recovery with painful sensory component Unilateral weakness, hyperreflexia
Miller-Fisher Syndrome (MFS) Sudden onset of weakness
Rare subtype, classic findings of areflexia, ataxia, and Changes in level of consciousness
ophthalmoplegia Asymmetry of weakness and paresthesia
Severe paresthesia without motor weakness
Acute Panautonomic Neuropathy Severe respiratory symptoms with limited motor
Very rare subtype affecting both sympathetic and weakness
parasympathetic systems Bladder or bowel dysfunction at the onset of
Recovery is slow and incomplete, with high mortality symptoms
and morbidity

bilaterally. Her cranial nerve exam was intact. Her motor peripheral myelin sheath. The commonly recognized
strength in triceps, biceps, and supinator were 5/5; deltoids variants are summarized i n Table 1.
and pronator were 4/5; wrist extensors and flexors were The patient in the case study provides many o f the
4/5 bilaterally; hip flexors, quadriceps, and hamstrings classic characteristics o f GBS, such as progressive
were 4/5 bilaterally; dorsiflexors and plantar flexors were development o f ascending symmetrical paresthesia,
3/5 bilaterally. Her sensation was intact on upper extremities pain, bilateral m o t o r weakness, areflexia, and ataxia.
but diminished to touch and pin prick on lower extremities Accurate diagnosis requires clues from the clinical his-
and abdomen. Her gait was ataxic, deep tendon reflex tory, such as onset, severity', and rate o f progression o f
( D T R ) 1 + and symmetrical on deltoid, biceps, brachio- the symptoms over hours to days. A careful n e u r o l o g i -
radialis and triceps; D T R was absent on knees and ankles. cal exammation m c l u d i n g the m o t o r strength, cranial
The patient's laboratory studies did not reveal any nerves, and reflexes is essential. Clinical manifestations
infectious process. H e r cerebrospinal fluid (CSF) studies are summarized i n Table 2.
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 Antecedent Infections
the diagnosis o f GBS. The disease can progress rapidly The history o f a prodromal respiratory infection should
and can be potentially fatal i f treatment interventions are raise suspicion for GBS, because in majority o f GBS
delayed. The patient was transported expeditiously to the patients, neuropathic symptoms are preceded w i t h a U R l
nearest hospital for admission to the intensive care unit. or enteric infection. History o f cough, fever, sore throat,
diarrhea, or any other types o f infections 2-3 weeks
DIAGNOSIS before the onset o f weakness should alert the clinician to
GBS is a heterogeneous syndrome w i t h several variant suspect GBS. Campylobacter jejuni, a bacteria commonly
forms affecting the peripheral nervous system as a result associated w i t h gastroenteritis, have been identified as the
o f an immune-mediated disturbance involving the most frequent antecedent pathogen o f GBS.'

644 The Journal for Nurse Practitioners - JNP Volume 8, Issue 8, September 2012
Vaccinations Table 3. Differential Diagnoses^^'^"
The evolution o f GBS after vaccination has been studied Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
extensively, as concerns about vaccine-induced GBS were S y m p t o m s similar t o GBS but progressing over a longer
initially raised after the 1976-77 influenza vaccination period ( > 8 weeks)
period.The landmark study done by Lasky et al ' Myasthenia Gravis (MG)
reported marginally increased risk o f GBS after influenza 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
vaccination during the first 6 weeks o f immunization. eyelids, face, s w a l l o w i n g

Subsequently, researchers investigated the relationship 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
between GBS and the influenza vaccinations and
Diplopia and/or ptosis, n o r m a l sensation and reflexes
reported that low relative risks were not statistically sig-
nificant.'"Extensive research is reported after the H l N l Anterior Horn Cell Abnormalities
S o m e similar features, but weakness pattern is different
mass vaccination i n 2009 and found no evidence o f an
Clinical signs of infection, i n c l u d i n g high cell count in
increased risk o f GBS after the seasonal influenza vaccine
cerebrospinal fluid
or the H l N l vaccination." Spinal Cord Disorders
Radiculopathy, unilateral m o t o r and sensory deficits
Diagnostic Studies Hyperreflexia, sharp sensory levels
CSF analysis w i t h evidence o f high protein and normal Pain aggravated w i t h activities

cell count support the diagnosis o f GBS. The value o f Intracranial Abnormalities
lumbar puncture is limited i n the early phase o f the dis- Change In level o f consciousness, severe headaches
Unilateral m o t o r and sensory deficits
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 to make the diagnosis on clinical grounds and promptly
cUnician to consider other diagnoses and investigate pos- refer to hospital/specialist care.*^''-*
sibility o f infectious process, such as leptomeningeal
malignancy. West N i l e virus infection, HIV, or Lyme dis- Differential Diagnosis
ease.'- I f the CSF results are normal, repeat testing is not Ascending symmetrical weakness progressing over hours
done typically. to days is the cardinal symptom o f GBS. I f the symptoms
Electrodiagnostic testing is done frequently to iden- are prolonged over 8 weeks, other diagnoses should be
tify the acute motor weakness caused by a peripheral considered. I f the weakness is asymmetric, clinicians
neuropathy. T h e test is helpful i n confirming the diagno- should consider a spinal or intracranial diagnosis instead
sis and differentiating the variants o f GBS. However, o f GBS. I f there is weakness w i t h intact reflexes, another
early in the disease process, the testing may be normal. diagnosis should be investigated. The predominant fea-
The features o f deniyelination i n GBS include slow con- tures o f Miller Fisher Syndrome, such as ophthalmo-
duction, temporal dispersion, and prolonged or absent plegia, areflexia, and ataxia, often mistakenly suggest
F-responses.^ Abnormal median and ulnar sensory brain stem infarction. A wide range o f neurological disor-
potentials w i t h spared sural potentials are seen i n the ders may mimic the symptoms o f GBS; most comnion
initial stages o f GBS.''The nerve conduction study is differentials are hsted i n Table 3.
considered a useful confirmatory test, w h i c h typically
demonstrates the finding o f reduced muscle action PATHOPHYSIOLOGY
potentials in chnically weak m u s c l e s . M a g n e t i c reso- The pathogenesis o f GBS has been widely studied but is
nance imaging ( M R I ) is typically performed to rule out still not completely understood.The proposed mecha-
infiltrative or structural causes o f weakness. Moreover, nism involves an antecedent infection leading to an auto-
M R I may reveal enhancement o f the affected nerve immune response reacting w i t h peripheral nerve
roots supportive o f GBS diagnosis. The hmitations o f components (Figure 1). Most o f the pathogens gains
these tests i n the early phase o f the disease, combined entry to the body through mucosal or gut epithehum
w i t h the urgency for early treatment, require clinicians and induce antibody production against specific ganglio- The Journal for Nurse Practitioners - JNP 645

Figure 1. Guillain-Barre Syndrome Pathogenesis.

Pathogenesis and recovery Disease-modifying factors

Campylobacterjejuni infection Bacterial factors

LOS biosynthesis cluster
Ganglioside mimicry of LOS

Immune response to LOS Host factors
Genetic polymorphisms
Immune status


Plasma cell
Cross-reactive antibodies
to nerve gangliosides

^ k h


Extent of nerve damage

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

Conduction dysfunction/block

Clinical prognostic factors

I Severity at onset
X ' Diarrhoea


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- Table 4. Autonomic Dysfunction^'^^
terial factors, such as the specificity o f lipo-ohgosaccha- Tachycardia, bradycardia
ride (LOS), and on host factors, such as genetic
Hypotension, hypertension
polymorphism and immune status.'"The presence o f
Facial flushing, anhidrosis, hyperhidrosis
antibodies leads to activation o f T cells and complements,
Constipation, paralytic ileus
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 exchange o f about 5 plasma volumes. The Cochrane
axons. The extent o f nerve damage varies, w i t h more Review'^ demonstrated that I V I g and PE are beneficial
damage seen in the intensely myelinated peripheral for accelerating the recovery o f GBS, i f given during the
nerves, causing motor and sensory weakness. first 4 weeks o f the disease, w i t h most benefit seen i f
The self-limiting nature o f the disease process given w i t h i n the first 2 weeks o f symptoms.
should be taken into account, symptoms improve once
the autoimmune inflammatory process is terminated, CORTICOSTEROIDS
and the Schwann cells reverse the process to rebuild the The role o f steroids i n GBS treatment has been widely
myelination o f the nerves. I n severe cases, the inflamma- studied, and researchers concluded that oral corticos-
tory process can lead to axonal disruption and teroids were not beneficial in GBS treatment and did not
permanent damage. recommend it as the first-line therapy."'This finding is in
contrast to the standard treatment for other demyelinat-
SUPPORTIVE CARE ing diseases for which favorable response is noted w i t h
Prevention o f life-threatening complications remains the steroid therapy. I n 1. o f the studies, the use o f intravenous
cornerstone o f supportive care. Progressive demyehnation corticosteroids in combination w i t h I V I g demonstrated a
o f the phrenic nerve, innervating the diaphragm, may lead short-term improvement i n clinical symptoms when
to acute respiratory muscle paralysis. Early detection o f res- compared to I V I g treatment a l o n e . L o n g - t e r m use o f
piratory failure and anticipation o f mechanical ventilation corticosteroids causes numerous side effects and may
are crucial to avoid emergency intubation and cardiopul- inhibit macrophage repair process, but short-term
monary arrest. Life-threatening episodes o f hemodynamic treatment did not result i n serious side effects.'^
instability related to autonomic dysfunction may occur in
GBS patients (Table 4).They should be admitted to the PAIN MANAGEMENT
hospital for close monitoring o f respiratory status, hemo- Neuropathic pain occurs i n large number o f patients
dynamic instability, and autonomic dysfunction. w i t h GBS and often requires recognition and treatment.
Nonsteroidal anti-inflammatory drugs (NSAIDs) may be
IMMUNOTHERAPY tried initially, but they often do not provide adequate
Immunotherapy should be initiated as soon as possible pain relief, as the origin o f pain is usually multifactorial.
w i t h high dose intravenous immunoglobuhn (IVIg) or Pain in the acute phase might be nociceptive related to
plasma exchange (PE).The mechanism o f action o f I V I g inflammation, whereas later in the course, pain may be
involves modulating the humoral response by suppressing related to degeneration o f sensory nerve fibers.'^ Early
autoantibody production.^ I V I g also blocks the binding recognition and treatment w i t h gabapentin or carba-
o f receptors on macrophages, suppressing the various mazepine are suggested to be effective i n treating neuro-
inflammatory mediators. The typical dose o f I V I g is pathic pain. Narcotic analgesics should be used w i t h
0.4g/kg per day for 5 days, started as a lower dose and caution as they precipitate ileus in the setting o f
increased to the m a x i m u m dosage based on patient toler- autonomic dysfunction.
ance. The goal o f PE is to remove circulating i m m u n e -
reactive antibodies, complements, and biological response PROGNOSIS
modifiers. The factors indicating poor prognosis include older age,
The treatment regimen depends on the disease sever- infection, prolonged hospital stay, need for mechanical
ity; typically, PE is given 5 times i n 2 weeks, for a total ventilation and intensive care, and poor upper extremity The Journal for Nurse Practitioners - J N P 647

Table 5. Complications^' should be considered in the differential diagnosis for
Respiratory failure, cardiac arrhythmias patients presenting to primary care setting with lower
Swallowing difficulties, aspiration pneumonia extremity numbness with antecedent history of upper
Deep vein thrombosis, foot drop and joint contractures
respiratory infection or gastroenteritis.The long-term
prognosis is dependent on early diagnosis and treatment
Bladder and bowel dysfunction, paralytic ileus
and knowledge o f prognostic factors can substantially
improve patient care. Biti
weakness." G B S patients may also exhibit pain, fatigabil-
ity', and functional impairments and may have permanent
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2. National Institute of Neurological Disorders and Stroke. Guillain-Barre
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most patients recover within 6-12 months, but some may Accessed July 16, 2012.
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comparison of Guillain Barre syndrome with multiple sclerosis in a
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vented with use o f fractionated or unfractionated heparin

4. Frenzen PD. Economic cost of Guillain-Barre syndrome in the United States.
Neurology. 2008;71{l):21-27.
and compression stockings.** Early initiation o f an indi- 5. Newswanger DL, Warren CR. Guillain-Barre syndrome. Am Fam Physician.
vidualized program for muscle strengthening and physical 6. Pithadia A, Kakadia N. Guillain-Barre syndrome. Pharmacol Rep.

therapy is essential to prevent complications (Table 5).

7. Davids HR, Oleszek JL, Cha-Kim A. Guillain-Barre syndrome. Accessed August 9, 2011.
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EDUCATION AND COUNSELING 9. Lasky T, Terracciano GJ, Magder L, et al. The Guillain -Barre syndrome and

Clearly, GBS management includes applying an improved

the 1992-1993 and 1993-1994 influenza vaccines. N Engl J Med.
understanding of the pathophysiology to individual 10. Kuitwaard K, Bos-Eyssen ME, Blomkwist-Markens PH, van Doom PA.
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tissues. Most patients develop secondary complications,

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.
and tailoring o f supportive care and education should be 12. Van Doom PA, Ruts L, Jacobs BC. Clinical features, pathogenesis, and
treatment of Guillain-Barre syndrome. Lancet Neurol. 2008;7(10):939-950.
based on their unique needs. T h e care of a G B S patient is 13. Winer JB. Guillain-Barre syndrome. Br Med J. 2008;337:227-231.

challenging for the health care team and the caregivers

14. Spalice A, Parisi P Papetti L, et al. Clinical and pharmacological aspects of
inflammatory demyelinating diseases in childhood: an update. Curr
because some symptoms can be devastating. G B S is a life NeuropharmacoL 2010;8(2):135-148.
15. Hughes RA, Raphael JC, Swan AV, van Doom PA. Intravenous
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cal and psychosocial well-being, transforming a healthy,

16. Bromberg MB, Carter O. Corticosteroid use in the treatment of
independent person into a critically ill and physically neuromuscular disorders: empirical and evidence-based data. Muscle Nerve.
helpless p e r s o n . ' ' T h e acute progression of motor weak- 17. Khan F, Pallant JF Ng L, Bhasker A. Factors associated with long-term
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quahty of hfe. 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.
Several neuromuscular disease organizations provide 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.
resources to assist w i t h community support networks. 2005;112(l):51-56.

T h e Guillain-Barre S y n d r o m e / C h r o n i c Inflammatory
Demyelinating Polyneuropathy Foundation
( is an excellent place to Sheiiy Sebastian, DNP, R.N, NP-C, is a luirsc practitioner in
get support, find up-to-date information, and seek the neurosurgery department at Baylor College of Medicine in
opportunities to network. Houston, TX, and can be reached at sherlysebast- In compliance with national ethical guide-
CONCLUSION lines, the author reports no relationships with business or
Even though significant advances have been made industry that would pose a conflict of interest.
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- 1565-4156/121/S see front matter
2012 American College of Nurse Practitioners
sis associated with high morbiditx' and mortality. GBS http://dx.doi.Org/10.1016/j.nurpra.2012.04.015

648 The Journal for Nurse Practitioners - J N P Volume 8, Issue 8, September 2012