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Guillain-Barré Syndrome – Third time’s the charm

 MAY 22ND, 2017  SREE NATESAN  CATEGORIES: PRACTICE UPDATES

Author: Sreeja Natesan, MD (@sreeja_natesan (https://twitter.com/sreeja_natesan) from

@dukeemergency (https://twitter.com/dukeemergency)– EM Attending Physician / Assistant
Program Director, Duke University Medical Center) // Edited by: Alex Koyfman, MD
(@EMHighAK (https://twitter.com/EMHighAK) – EM Attending Physician, UT Southwestern
Medical Center / Parkland Memorial Hospital) and Manpreet Singh, MD (@MPrizzleER
(https://twitter.com/mprizzleer) – Assistant Professor of Emergency Medicine / Department
of Emergency Medicine – Harbor-UCLA Medical Center)

Case: 60 year-old male PMH of Htn, DM, presents to the ED with complaints of generalized
weakness and fatigue.  His family states you are the third provider he has seen in the last week. 
He was seen by the primary care doctor for fever and cough 5 days prior and started on
Azithromycin for bronchitis and was seen at the Urgent care for fatigue and weakness 1 day
prior to presentation where he was treated with IVF.  Despite antibiotics and IVF, he reports
continued symptoms of generalized weakness.  He denies focal weakness, headache or neck
stiffness and has an otherwise negative ROS.  Vital signs are within normal limits on arrival.  PE
reveals a normal exam except for the neurologic component which identi es decreased strength
in bilateral LE with are exia.  What is your next step?

Background:
How common is GBS? 
Guillain-Barré Syndrome (GBS) has an estimated incidence of 1 to 4 per 100,0001 making it the
most common cause of acute generalize paralysis since the virtual elimination of
poliomyelitis2,3.  GBS is a commonly missed diagnosis in the ED which has the potential to
progress to signi cant morbidity1.

How often is it missed?

How often is it missed? 
Diagnosis of early acute GBS is often missed due to the failure to recognize key features
essential in the diagnosis of the disease.  This is often due to the minimizing the paresthesia,
discounting the existence of the complaint, not being vigilant to check deep tendon re exes, or
misinterpreting the complaints of leg, back, or sciatic pain2.  A retrospective case series revealed
that only 25% of patients were accurately diagnosed with GBS during their rst visit with an
average of 2 visits need to make the correct diagnosis1.

De nition
Guillain-Barré Syndrome is an in ammatory peripheral neuropathy. Up to 88% of patients
affected by GBS have a prodromal infection4.  As an aberrant response to a precipitating
infection, a primarily lymphocytic T cell mediated and macrophage mediated response results in
demyelination, although the exact mechanism is still under investigation2.  It is however become
clearer that infection can induce antibodies that then cross react with neural antigens which
leads to the resultant in ammatory neuropathy5.

It can present with paresthesia, ascending weakness, and are exia one to six weeks after
infectious prodrome4, although there are variable presentations within the 4 subtypes as seen in
the box below. Miller-Fischer syndrome accounts for 5% of the cases and includes ataxia and
ophthalmoplegia in addition to the are exia1.

From: https://www.uspharmacist.com/article/guillain-barre-syndrome

The most common infections are from cytomegalovirus and Campylobacter jejuni1,2.  There are
4 main phases of the disease process including:

lb h i ii i f i ill d h f l
 1. Interval between the inciting infection illness and the onset of neuromuscular symptoms
2. Progressive weakness lasting less than one month
3. Plateau/nadir
4. Recovery

The onset of symptoms may vary although there is a rapidly progressive form that can result in
quadriplegia and respiratory failure within 48 hours1 and is associated with poorer outcomes5.

From: https://www.ahcmedia.com/articles/12128-key-neuromuscular-junction-disorders-
and-peripheral-neuropathies-for-the-emergency-physician

Important items in history and physical examination

History pearls
History is crucial in the diagnosis of GBS.  The disease process is occur 5-7 days after an
infection illness6.  Two thirds of cases of GBS follow an infection, typically viral but can include
such pathologic agents such as HIV, Epstein Barr virus, and Cytomegalovirus2.  Enteritis from
Campylobacter jejuni has been identi ed as an important etiology2, thus a history of fever,
crampy abdominal pain with nausea/vomiting/watery-diarrhea or bloody diarrhea would be
important to elicit from the patient.  Infection with M pneumoniae precedes about 5% of cases of
GBS5.  In a small population, GBS can also occur in the presence of an underlying systemic
disease process such as Hodgkin’s lymphoma, systemic lupus erythematosus, sarcoidosis, or a
recently acquired HIV infection2.

Onset of symptoms of weakness within the last 24 hours should raise concerns for GBS,
Myasthenia gravis, tick paralysis, stroke, or acute porphyric neuropathy6.

The chief complaint may center on complaints of pain or paresthesia/weakness3.  The course
can begin with ne paresthesia in the toes or ngertips that is followed within days by leg
weakness2.  Initially, the weakness may be proximal, distal, or both5 and typically ascends from
the lower extremities upward symmetrically.  Patients will often complain of weakness that
makes it dif cult to walk or climb stairs.  Pain is a common complaint, described as aching in the
large muscle of the upper legs, anks or back or as bilateral sciatica pain2.

 Physical exam pearls

Focus should be aimed at the evaluation for weakness noted as symmetric limb weakness,
greatly diminished or absent tendon re exes, and minimal loss of sensation despite
paresthesia2.  It is imperative to do a careful neurologic exam including strength and evaluation
of re exes to avoid missing this diagnosis4.  The facial nerves are often affected, although to a
lesser extent, can also involve the bulbar and ocular motor nerves5.  One third of patients may
present with bilateral facial weakness2.  Severe cases may present with respiratory depression,
autonomic dysfunction, or eye movement weakness.  Signs of autonomic involvement would
include: urine retention, ileus, sinus tachycardia, hypertension, cardiac arrhythmia, and postural
hypotension5.

From: http://www.natural-health-news.com/guillain-barre-syndrome-causes-symptoms-
diagnoses-and-treatment/

Diagnostic Considerations:
Nerve conduction abnormalities occur earlier and more frequently than the elevation of protein
in cerebrospinal uid due to the re ecting demyelination and thus electrophysiology studies
such as electromyography (EMG) are the most sensitive and speci c diagnostic tool2 .  However,
this is usually not feasible to attain in the ED1 raising use of a thorough history, physical exam,
and lumbar puncture as the tools to help a clinician correctly make this diagnosis.

 Cerebrospinal uid Studies

Con rmatory workup involves a lumbar puncture (LP) in which the CSF is evaluated for
albuminocytologic dissociation without an elevated cell count (high protein (>45) and low
WBC count (<10))1.  The LP results that is consistent with GBS includes normal opening
pressure, few or no cells, and a protein concentration that is elevated2.  A raised CSF protein
concentration is found in up to 80% of patients although it is important to know that in the rst
few days of the illness it may be normal5.

 Guillain Barré Syndrome (GBS) Diagnostic Criteria

From: https://www.slideshare.net/drpramodkrishnan/gb-syndrome

Differential diagnosis of acute accid paralysis5

Management
The rst priority in any patient arriving to the Emergency department should focus on ABCs and
a quick review of life threatening causes of weakness as seen in the following box6.

Acute Life-threatening Causes of Weakness6

Myasthenia gravis
Guillain-Barre Syndrome
Botulism
Adrenal insuf ciency/hypermagnesemia
Organophosphate poisoning
Carbon monoxide poisoning
Hypokalemia
Cerebrovascular accident
Seizure
Spinal cord compression
Encephalopathy
Sepsis

Intubation/RSI pearls
Weakness of the respiratory muscles can cause patients with GBS to require arti cial ventilation
in up to 25% of patients1,5.  Regular monitor of vital capacity can be useful in determining if
prophylactic intubation is necessary5.  Early intubation is recommended for patients for airway
protection with guidance using the 20-30-40 rule (given in the following box)6.  Other potential
indications would be inability to lift head or shoulders, inability cough, rapid onset of symptoms6.

20-30-40 Rule for Intubation in GBS

Forced vital capacity < 20ml/kg
Max inspiratory pressure <30cm H20
Max expiratory pressure <40cm H20
Regarding RSI: succinylcholine should be avoided due to signi cant risk of hyperkalemia, which
results due to the upregulation of muscle acetylcholine receptors6,7.

Medication Considerations
14
Plasma exchange versus immunoglobulin (IVIG) therapy are the mainstay therapy1,4 and are
more ef cacious when started during the rst 2 weeks of symptoms6. However since continued
research including randomized controlled trials have showed similar ef cacy to plasma
exchange, IVIG has replaced plasma exchange in treatment of severe GBS due to convenience5. 
CSF analysis should be done before initiating IVIG as the implementation of IVIG may cause an
aseptic meningitis5. The American Academy of Neurology practice parameter does recommend
either plasma exchange or IVIG for treatment of GBS patients who have lost the ability to walk,
although best practice is still undecided5. Corticosteroids are no longer considered useful in the
therapy for GBS2.

From: http://www.nature.com/nrneurol/journal/v10/n8/full/nrneurol.2014.121.html

Disposition
Hospitalization for observation for at least several days should occur for almost all patients with
Guillain-Barré Syndrome2.  Mild cases that include only mildly distal paresthesia or mild limb
weakness may not warrant therapy, although it is suggested to wait approximately two weeks
before concluding that there will be no further progression2.  Intensive Care admission should
be considered in patient with cardiovascular dysautonomia or those patient with a vital capacity
that is <18ml/kg body weight or is rapidly declining2.

Prognosis
Weakness typically stops advancing in one to four weeks, with a nadir by 2 weeks, eventually
reaching a plateau phase by 4 weeks in nearly all cases2,5.  A typical patient during this process
will become bedridden due to the weakness with distal paresthesia, incomplete bilateral
weakness of facial muscles, dif culty swallowing and half of the predicted vital capacity2.  There
are varying degrees of the severity and identi cation of this is crucial to ensure the best
outcomes.  In both adult and children, the severity of the disease at the nadir has been identi ed
as an adverse prognostic factor (expressed as being bed bound or requiring arti cial
ventilation)5.

Prompt treatment has been associated with good long-term outcomes including an 85% full
recovery6.  Between 4-15% of patients die despite medical therapy, from largely avoidable
complications such as sepsis, adult respiratory distress syndrome, pulmonary embolism, and
dysautonomia leading to cardiac arrest2 with some references stating up to 20% being
permanently disabled5.  Minor residual de cits can be seen in up to 65 percent such as distal
numbness or foot drop although this does not impair daily life2.   Disabling weakness, imbalance
or sensory loss occur in 5 to 10 percent2.  Poorer outcomes are seen in patients who had rapid
onset of symptoms5.  Patients who at the nadir of 2 weeks are still able to walk, are likely to
improve with or without treatment with no residual de cit5.  Multi-disciplinary rehabilitation is
essential in the recovery of GBS patients.

The illness tends to be less severe in children than adult2 with recovery being more rapid and
complete5.  Also, if this occurs during pregnancy, it typically occurs during the third trimester or
post-partum and does not affect the fetus suggesting that the maternal IgG does not cause nerve
in ammation or demyelination2.

Pearls and Pitfalls:

Guillain-Barre Syndrome is a dif cult diagnosis to make and an accurate history and physical
examination including re exes and ambulation of the patient can help to identify this life-threatening
diagnosis
LP results will reveal a normal opening pressure, elevated protein level with no cells
Intubation consideration: remember the 20-30-40 rule and avoid succinylcholine for RSI
Management options: IVIG or plasmapheresis should be considered in patients with GBS. They should
be monitored closely in the hospital for progression.
Steroids have no role in treatment of GBS.

Case Resolution
The patient was admitted to neurology step down and had progression of his disease resulting in
intubation the next morning due to respiratory depression.  IVIG was started in the intensive
care unit and the patient’s symptoms stabilized.  He was eventually extubated and transferred to
the oor.  He was transitioned to a rehabilitation facility to help with physical therapy and
strength training.
References/Further Reading
1. Noto, A., & Marcolini, E. (2014). Select Topics in Neurocritical Care. Emergency Medicine Clinics of
North America, 32(4), 927-938.
2. Ropper A.H.: The Guillain-Barre syndrome. N Engl J Med 1992; 326: pp1130-1136.
3. McGillicuddy D.C., Walker O., Shapiro N.I., et al: Guillain-Barre syndrome in the emergency department.
Ann Emerg Med 2006; 47: 390-393.
4. Sheridan, J. (2010). Atypical Guillain-Barre in the emergency department. West J of Emerg Med, 11(1),
80-82.
5. Ganti, L., & Rastogi, V. (2016). Acute Generalized Weakness. Emergency Medicine Clinics of North
America, 34(4), 794-809.
6. Hughes RAC, Cornblath DR. Guillain-Barre Syndrome. Lancet 2005; 366: 1653-66.
7. Roppolo L.P., and Walters K.: Airway management in neurological emergencies. Neurocrit Care 2004; 1:
405-414.

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