Brief
in
Guillain-Barré Syndrome
Aimee Chung, MD,* Melissa Deimling, MD†
*Department of Medicine-Pediatrics, Duke University Medical Center, Durham, NC
†
Latham Pediatrics, Community Care Physicians, Latham, NY
AUTHOR DISCLOSURE Drs Chung and
Deimling have disclosed no financial
relationships relevant to this article. This
commentary does not contain a discussion
of an unapproved/investigative use of a
commercial product/device.
Guillain-Barré Syndrome. Yuki N, Hartung
HP. N Engl J Med. 2012;366(24):2294–2304
Guillain-Barré Syndrome. Hughes RA,
Cornblath DR. Lancet. 2005;366(9497):
1653–1666
Clinical Features, Pathogenesis, and
Treatment of Guillain-Barré Syndrome. van
Doorn PA, Ruts L, Jacobs BC. Lancet Neurol.
2008;7(10):939–950
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Guillain-Barré syndrome (GBS) is a peripheral nervous system disorder that
presents as a rapidly progressive, ascending, flaccid paralysis with diminished or
absent reflexes. The disease is often triggered by an infectious process, and it
affects children of all ages. Once considered a single disease, many subtypes have
been identified based on the neuronal components involved. Important subtypes
to consider include acute inflammatory demyelinating polyradiculoneuropathy,
which targets segments of the myelin sheath. Other subtypes include acute motor
axonal neuropathy and acute motor and sensory axonal neuropathy, both of which
target the neuronal axon. Miller Fisher syndrome (ataxia, areflexia, and ophthalmoplegia)
falls under the GBS categorization as well, although it usually involves minimal to
no weakness. With the eradication of poliomyelitis in the developed world, GBS is
now the most common cause of acute flaccid paralysis in infants and children,
although still rare in children younger than 2 years. The incidence in Western
countries ranges from 0.89 to 1.89 cases per 100,000 person-years. With every
10-year period after the first decade after birth, there is a 20% increase in
incidence, with males more affected than females.
Although poorly understood, the mechanism of GBS is likely related to an
immune-mediated trigger, such as an infection, that affects peripheral nerve
components due to molecular mimicry. Classically, this trigger is a gastrointes-
tinal or upper respiratory tract illness within 4 weeks of presentation. Possible
viral agents include cytomegalovirus, Epstein-Barr virus, influenza, and human
immunodeficiency virus, and bacterial triggers include Mycoplasma, Haemophilis,
and, most commonly, Campylobacter jejuni, which accounts for 20% to 30% of US
and European cases. Although rare, vaccination, surgery, trauma, transplant,
lymphoma, and systemic lupus erythematosus have also been associated with GBS.
The differential diagnosis of the presenting features of GBS (flaccid paralysis,
areflexia, or a combination) is extensive and may include pathology of the
cerebellum, spinal cord, peripheral nerves, and musculature. Conditions that
may have similar features include acute cerebellar ataxia, transverse myelitis,
spinal cord compression, poliomyelitis, tick-borne paralysis, myasthenia gravis,
myositis, and additional myopathies. Guillain-Barré syndrome is a clinical diag-
nosis, and progressive motor weakness of more than 1 limb and areflexia or
marked hyporeflexia are required. Features strongly supporting the diagnosis
include progression of onset over several days to less than 4 weeks, symmetri-
cal involvement, painful onset, mild/absent sensory symptoms, cranial nerve
involvement, autonomic dysfunction, absence of fever, and recovery 2 to 4 weeks
after the onset of peak or plateauing of symptoms. The most useful diagnostic
tests are cerebrospinal fluid (CSF) studies, electromyography, and, less specifi-
cally, magnetic resonance imaging (MRI). Classic CSF findings show an elevated
protein level (albuminocytologic dissociation), along with a normal cell count with
Vol. 39 No. 1 JANUARY 2018 53
 lymphocytic predominance. Electromyography results may long-term recovery regardless of subtype. The recurrence
show conduction abnormalities depending on the GBS risk is estimated to be 2% to 5%.
subtype. Last, MRI findings, although nonspecific, may
show gadolinium enhancement of the spinal nerve roots.
COMMENTS: Although it is fortunate that children tend to
For initial management, patients require close monitor-
experience less severe disease than adults, I am struck by the
ing of motor, autonomic, and respiratory function in the
variability of presentations. Although usually a monophasic
inpatient setting. Immunotherapy with intravenous immu-
disease, in some patients it may reoccur, with a mean interval
noglobulin (IVIG) or plasma exchange is the preferred
to reoccurrence of 7 years. Early and timely diagnosis is im-
treatment modality but should be reserved for patients with
portant because studies have suggested improved outcomes
severe GBS. Severe symptoms include progressive muscle
when treatment is initiated within the first 2 weeks. Although
weakness, respiratory compromise, bulbar symptom devel-
in most patients the maximal weakness presents in the first 2
opment, or the inability to walk unassisted. Although there
weeks, in some it may take up to 4 weeks, so it is important to
are no clear inferiority or superiority outcome trials, IVIG
have GBS in your differential diagnosis. The elevated protein
is typically selected over plasma exchange due to ease of
level in the CSF may not initially be present but is present in
administration and greater availability. The total dose of
approximately 50% of patients in the first week and in greater
IVIG in children is 2 g/kg, given as 1 g/kg for 2 days or 400
than 90% of patients after the second week of symptoms. Up
mg/kg for 5 days, usually as a single treatment course. The
to 20% of patients (when including adults) remain with some
proposed mechanism of action of IVIG is suppression of the
disability. Although much progress has been made, more
inflammatory and immune-mediated responses. In patients
research is needed. Active areas of research include assessing
with mild disease or nonprogressive symptoms, treatment
treatment effectiveness in patients with more mild disease and
may be supportive. Once stabilized, physical, occupational,
the best strategies to treat autonomic dysfunction and related
and speech therapies should be initiated early to restore and
fatigue noted in patients with GBS.
maintain function. Mortality is estimated to be 3% to 4%.
Children typically have a better prognosis than adults, and in – Janet R. Serwint, MD
previous studies, greater than 80% of children had excellent Associate Editor, In Brief

Additional Resources for Pediatricians

AAP Textbook of Pediatric Care, 2nd Edition
• Chapter 260: Guillain-Barré Syndrome - https://pediatriccare.solutions.aap.org/chapter.aspx?sectionId¼135886435&bookId¼1626
Point-of-Care Quick Reference
• Guillain-Barre Syndrome - https://pediatriccare.solutions.aap.org/content.aspx?gbosid¼311264
For a comprehensive library of AAP parent handouts, please go to the Pediatric Patient Education site at http://patiented.aap.org.

Correction
An omission on screening guidelines appeared in the December 2017 review “Postpartum Depression: What Do Pediatricians Need
to Know?” (Sriraman NK, Pham D, Kumar, R. Pediatrics in Review. 2017;38(12):541–551). According to current recommendations, screening
for postpartum depression should take place at the 1-, 2-, 4-, and 6-month well-child visits. The online article has been updated to reflect
this recommendation, and a correction was published online. The journal regrets the error.

54 Pediatrics in Review
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 Guillain-Barré Syndrome
Aimee Chung and Melissa Deimling
Pediatrics in Review 2018;39;53
DOI: 10.1542/pir.2017-0189

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 Guillain-Barré Syndrome
Aimee Chung and Melissa Deimling
Pediatrics in Review 2018;39;53
DOI: 10.1542/pir.2017-0189

The online version of this article, along with updated information and services, is
located on the World Wide Web at:
http://pedsinreview.aappublications.org/content/39/1/53

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