Pediatric Neurology 47 (2012) 91e96

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Pediatric Neurology
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Original Article

Clinical Variants of Guillain-Barr Syndrome in Children

Jainn-Jim Lin MD a, b, Shao-Hsuan Hsia MD b, Huei-Shyong Wang MD a, Rong-Kuo Lyu MD c,
Min-Liang Chou MD a, Po-Cheng Hung MD a, Meng-Ying Hsieh MD a, Kuang-Lin Lin MD a, *
a

Division of Pediatric Neurology, Chang Gung Childrens Hospital and Chang Gung Memorial Hospital, College of Medicine, Chang Gung University, Taoyuan,
Taiwan
b
Division of Pediatric Critical Care and Division of Emergency Medicine, Chang Gung Childrens Hospital and Chang Gung Memorial Hospital, College of
Medicine, Chang Gung University, Taoyuan, Taiwan
c
Department of Neurology, Chang Gung Childrens Hospital and Chang Gung Memorial Hospital, College of Medicine, Chang Gung University, Taoyuan,
Taiwan

article information

abstract

Article history:
Received 28 March 2012
Accepted 21 May 2012

Guillain-Barr syndrome is characterized by acute progressive weakness, areexia, and

maximal motor disability that occur within 4 weeks of onset. Various clinical subtypes have
been described since the original description of the syndrome. This study aimed to identify
characteristics of clinical variants of Guillain-Barr syndrome through retrospective review
of cases in Chang Gung Childrens Hospital from 2000-2010. Forty-three Guillain-Barr
syndrome patients were evaluated based on clinical presentations and an electrodiagnostic
study. The most frequent variant of Guillain-Barr syndrome was demyelinating polyneuropathy (67.4%), followed by acute axonal neuropathy (7.0%), Miller Fisher syndrome
(7.0%), Bickerstaff brainstem encephalitis (7.0%), pharyngo-cervical-brachial variant (4.7%),
and polyneuritis cranialis (4.7%). Follow-up revealed that 35 recovered satisfactorily, eight
were persistently disabled, and none died during hospitalization. At the earliest stage,
differentiating clinical variants from typical Guillain-Barr syndrome was difcult. Children
with clinical variants of Guillain-Barr syndrome are more likely to manifest rapid onset
from disease onset to nadir, increasing the severity of disability, cranial nerve involvement,
urine incontinence, respiratory impairment, and need for ventilator support than in typical
Guillain-Barr syndrome.
2012 Elsevier Inc. All rights reserved.

Introduction

Guillain-Barr syndrome is characterized by acute

progressive weakness, areexia, and maximal motor
disability that occur within 4 weeks of onset. It is an acute,
postinfectious, immune-mediated peripheral neuropathy
with highly variable clinical courses and outcomes [1-3].
Various clinical subtypes have been described since the
original description of the syndrome [4-7]. Variant forms
are characterized by their localized or regional involvement
of the peripheral and autonomic nerves. Because no single
* Communications should be addressed to: Dr. Lin; Division of Pediatric Neurology; Chang Gung Childrens Hospital; 5 Fu-Shin Street;
Kwei-Shan, Taoyuan 333, Taiwan.
E-mail address: lincgh@adm.cgmh.org.tw
0887-8994/$ - see front matter 2012 Elsevier Inc. All rights reserved.
doi:10.1016/j.pediatrneurol.2012.05.011

clinical or serologic marker exists for Guillain-Barr

syndrome, the diagnosis is based on consistent clinical,
laboratory, and neurophysiologic ndings, along with the
exclusion of other conditions mimicking the disorder [5].
Guillain-Barr syndrome is recognized as a heterogeneous disorder with various clinical manifestations. Recent
neurophysiologic and pathologic ndings have led to its
reclassication [4,8,9], expanding the clinical and pathologic spectrum from classic acute inammatory demyelinating polyneuropathy and axonal variants to several clinical
variants (i.e., Miller Fisher syndrome, Bickerstaff brainstem
encephalitis, pharyngo-cervical-brachial variant, polyneuritis cranialis, and acute sensory neuropathy) [4-9].
Failure to recognize these Guillain-Barr syndrome variants
can lead to erroneous diagnoses, inappropriate treatment,
and signicant morbidity.

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J.-J. Lin et al. / Pediatric Neurology 47 (2012) 91e96

The present study aimed to describe the clinical manifestations, laboratory and imaging ndings, treatment, and
prognosis in children manifesting clinical variants of
Guillain-Barr syndrome. A diagnostic owchart for Guillain-Barr syndrome variants is described, with emphasis
on the early recognition of Guillain-Barr syndrome.
Methods
Diagnoses of acute Guillain-Barr syndrome were mainly based on
symptomatology and on supportive ancillary data, including the results
of cerebrospinal uid, electrophysiologic, or imaging evaluations [1-3].
Previously healthy children aged <18 years with a discharge diagnosis of
Guillain-Barr syndrome were included, but not those with chronic
inammatory demyelinating polyneuropathy or conditions known to
cause acute polyneuropathy, and with a previous underlying disease
such as a history of autoimmune disease, previous neurologic insult, or
a progressive neurologic disorder.
The electrodiagnostic study and medical records of patients with
Guillain-Barr syndrome admitted to Chang Gung Childrens Hospital
between January 2000 and June 2010 were retrospectively reviewed.
Forty-three children were enrolled after being examined by a neurologist. Information collected included age at presentation, sex, subtype of
classication, preceding infection (respiratory or gastrointestinal tract),
time from onset of signs to nadir, cranial nerve involvement
(e.g., impaired gag reex, dysphagia, or dysarthria), autonomic
dysfunction (e.g., exceptional blood pressure uctuations or unexplained
cardiac arrhythmia), peak clinical severity, neuroimaging, specic
therapy (i.e., intravenous steroids, immunoglobulin, or plasma
exchange), length of hospital stay, and prognosis.
Guillain-Barr syndrome variants were initially divided into two
groups according to clinical presentation, as either the classic ascending
form or atypical presenting forms. The typical ascending form of
Guillain-Barr syndrome was characterized by progressive, symmetric,
ascending accid paresis with areexia [1-3]. A diagnostic owchart is
presented in Fig 1. The atypical presentation group of Guillain-Barr
syndrome was characterized by localized or regional involvements of the
motor and sensory axons of the peripheral nerves and the autonomic

nervous system [4-7]. In the typical ascending group, two subtypes were
classied according to electrodiagnostic study: (1) the demyelination
forms (e.g., acute inammatory demyelinating polyradiculoneuropathy)
and (2) the axonal forms, which included acute motor-sensory axonal
neuropathy and acute motor axonal neuropathy. In the atypical
presentation group, two subtypes were classied according to atypical
presentation and neurophysiologic ndings: (1) prominent cranial nerve
involvement, which included Miller Fisher syndrome, Bickerstaff brainstem encephalitis, pharyngo-cervical-brachial, and polyneuritis cranialis; and (2) others, which included acute pandysautonomia and acute
sensory neuropathy.
To determine whether the clinical presentations and outcomes of
clinical Guillain-Barr syndrome variants differed from typical
Guillain-Barr syndrome, acute inammatory demyelinating polyradiculoneuropathy was dened as typical Guillain-Barr syndrome, and
others (including the axonal forms and atypical presentations) were
dened as clinical variants. Peak clinical severity was quantied by
degree of maximal upper limb weakness, according to an ordinal scale,
i.e., 0, normal; 1, weak, but able to lift arms off bed; 2, icker of movement; 3, no movement [10]. Outcomes at 1 year after disease onset were
assessed. Functional outcomes were ranked according to our adaptation
of the scale by Hughes et al. [11], i.e., 0, healthy; 1, minor signs, able to
run; 2, able to walk >5 m without assistance, but unable to run; 3, able to
walk >5 m with assistance; 4, bedbound or chairbound; 5, requiring
assisted ventilation for at least part of the day; and 6, dead. A favorable
outcome was dened as functional independence and the ability to
ambulate independently (Hughes score of 0-2) [10]. Our hospitals
Institutional Review Board approved this study.
Statistical analysis

The clinical, biochemical, and electrophysiologic features and

outcomes of clinical Guillain-Barr syndrome variants were compared
with those of typical Guillain-Barr syndrome. Clinical factors obtained
upon admission were analyzed for possible differences between the two
groups. These factors included age, sex, preceding infection, GuillainBarr syndrome subtype, time from onset of signs to admission, cranial
nerve involvement, dysautonomia, peak clinical severity, neuroimaging,
and specic therapy. The associations between each of these variables
and outcomes were also examined.

Figure 1. A diagnostic owchart for Guillain-Barr syndrome and variants. The typical group includes (1) acute inammatory demyelinating polyradiculoneuropathy (AIDP), and (2) acute motor-sensory axonal neuropathy (AMSAN) and acute motor axonal neuropathy (AMAN). The atypical group
includes (1) Miller Fisher syndrome (MFS), Bickerstaff brainstem encephalitis (BBE), pharyngo-cervical-brachial variants (PCB), and polyneuritis cranialis
(PN), and (2) acute pandysautonomia and acute sensory neuropathy (ASN). CSF, cerebrospinal uid; MRI, magnetic resonance imaging.

J.-J. Lin et al. / Pediatric Neurology 47 (2012) 91e96

Table 1. Classication of Guillain-Barr syndrome according to clinical presentations and electrodiagnostic study in 43 children

Type
Typical ascending forms
(1) Demyelination forms of GBS: AIDP
(2) Axonal forms of GBS
Acute motor-sensory axonal neuropathy
Acute motor axonal neuropathy
Atypical presentations
(1) Prominent cranial nerve involvement
Miller Fisher syndrome
Bickerstaff brainstem encephalitis
Pharyngo-cervical-brachial variant
Polyneuritis cranialis
(2) Other
Acute pandysautonomia
Acute sensory neuropathy

Number
(n 43)

Percentage
(%)

29
3
1
2

67.4
7.0
2.3
4.7

11
4
3
2
2

25.6
9.3
7.0
4.6
4.6

0
0

0
0

Abbreviations:
AIDP Acute inammatory demyelinating polyradiculoneuropathy
GBS Guillain-Barr syndrome

Statistical analysis was performed using the SPSS version 12.0

statistical software (SPSS, Inc., Chicago, IL). Univariate factors predictive
of outcomes (good or poor) were assessed using the Mann-Whitney
U test for continuous variables, and the Fisher exact test for categorical
variables. P < 0.05 was considered statistically signicant. All statistical
tests were two-tailed.

Results
Demographic data

Forty-three patients with Guillain-Barr syndrome,

based on clinical and electrophysiologic data, aged 1 year
and 8 months to 18 years (mean
standard deviation, 7.88

5.31 years), were enrolled. Thirteen (30.2%) girls and 30
(69.8%) boys were included. Nineteen (44.2%) were aged <4
years old, 11 (25.6%) were 5-8 years old, one (2.3%) was in
the age group of 9-12 years, and 12 (27.9%) were >13 years
old. The highest incidence of Guillain-Barr syndrome
occurred in the group aged <4 years. Approximately two
thirds of the patients were aged <8 years.
Classication

Guillain-Barr syndrome variants according to clinical

presentations and electrodiagnostic study are listed in
Table 1. In the classic ascending group, 29 patients (67.4%)
manifested acute inammatory demyelinating polyradiculoneuropathy, two (4.7%) manifested acute motor
axonal neuropathy, and one (2.3%) manifested acute motorsensory axonal neuropathy.
In the atypical presentation group, 11 patients (25.6%)
manifested prominent cranial nerve involvement, including
four (9.3%) with Miller Fisher syndrome, three (7.0%) with
Bickerstaff brainstem encephalitis, two (4.7%) with the
pharyngo-cervical-brachial variant, and two (4.7%) with
polyneuritis cranialis. No signicant differences were
evident between age and classication.
Clinical manifestations

The most common initial sign was limb weakness,

documented in 100 (100%) patients, followed by muscle

93

pain or soreness (n 19, 44.2%) and paresthesia (n 18,

41.9%). The mean time from onset of illness to nadir in all 43
patients was 5.69 days (range, 1-20 days), divided into 6.79
days (range, 1-20 days) in typical Guillain-Barr syndrome
and 3.50 days (range, 1-10 days) in the clinical variant
group. The time from onset to illness to nadir was shorter in
the clinical variant form group than in the classic ascending
group, but no signicant difference was evident between
these two groups (P 0.014). The mean disability score at
nadir
the standard deviation was 0.83
0.80 in typical
Guillain-Barr syndrome, and 1.93
0.92 in the clinical
variant group according to peak clinical severity.
We scored 28 patients at grade 3 or more during hospitalization according to the functional grading scale of
Hughes et al. [11]. The score in typical Guillain-Barr
syndrome was 2.76
0.91, and score in the clinical variant
group was 4.00
0.96, according to the scale of Hughes
et al. [11]. Signicant differences were evident between
these two groups in terms of mean disability score and
Hughes scale at nadir (P 0.004 and 0.004, respectively).
Other signs included cranial nerve involvement in 17
(39.5%), autonomic dysfunction in 13 (30.2%), urinary
incontinence in 11 (25.6%), and headache/dizziness in nine
(20.9%). Children in the clinical variant group were more
likely to manifest cranial nerve involvement and urinary
incontinence than those with typical Guillain-Barr
syndrome (P  0.001, and < 0.001, respectively). Respiratory
signs, including chest tightness or dyspnea, were evident in
10 patients (23.3%). Seven (16.3%) required ventilator
support during hospitalization. In the clinical variant group,
the numbers of patients with respiratory signs (seven of 14
children) and receiving ventilator support (ve of 14 children) were signicantly higher than those with typical
Guillain-Barr syndrome (three of 29 children and two of 29
children, P 0.007 and 0.028, respectively). No patient died
during hospitalization. A comparison of demographic
features between the classic ascending form group and the
clinical variant group is presented in Table 2.
Seasonal preponderance and preceding infection

Nine patients developed Guillain-Barr syndrome during

spring (March-May), 12 during summer (June-August), 10
during fall (September-November), and 12 during winter
(December-February). The clinical variant group mostly
involved summer and winter, and a signicant difference
was evident between these two groups. Four patients (9.3%)
exhibited no known triggering event 4 weeks previous to
the onset of Guillain-Barr syndrome. Of the 39 patients
(90.7%) with triggering events, 28 patients exhibited these
events within 2 weeks before the onset of Guillain-Barr
syndrome, and 11 patients exhibited these events between
2-4 weeks before the onset of Guillain-Barr syndrome.
Upper respiratory infection (86%, 37/43) comprised the most
frequent trigger, followed by acute gastroenteritis (4.7%,
2/43). Moreover, neither a family history of this disease nor
illness in other members of the household before or during
the onset of signs suggested a possible neuropathic virus or
other infection. None had received vaccinations.
Among the 37 patients with upper respiratory tract
infection in the Guillain-Barr syndrome group, 12 were
infected with Mycoplasma pneumoniae (32.4%), ve with

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J.-J. Lin et al. / Pediatric Neurology 47 (2012) 91e96

Table 2. Comparison of demographic features between typical Guillain-Barr syndrome and atypical variants

Sex
Male
Female
Age (years)
<8
9-18
Onset of illness to nadir in number of days
(mean, range)
Preceding illness
URI
AGE
None
Seasonal occurrence
December-February
March-May
June-August
September-November
Initial signs
Peak clinical severity
0
1
2
3
Mean
S.D.
Functional grading of disease
2
3
4
5
Mean
S.D.
Cranial nerve involvement
Autonomic dysfunction
Urine incontinence
Headache/dizziness
Respiratory signs
Ventilator support

Typical GBS
(n 29)

Atypical Variants
(n 14)

Odds Ratio

95% CI

P Value

18
11

12
2

0.164

20
9
6.79 (1-20)

9
5
3.50 (1-10)

0.759

25
1
3

12
1
1

0.826

6
7
6
10

6
2
6
0

0.035*

0.014*

11
13
4
1
0.83
0.80

1
3
6
4
1.93
0.92

0.004*

14
10
3
2
2.76
0.91
6
8
2
6
3
2

1
3
5
5
4.00
0.96
11
5
9
3
7
5

0.004*

14.05
1.46
24.30
1.04
8.67
7.50

2.95-66.96
0.37-5.70
3.99-147.74
0.22-4.98
1.77-42.46
1.23-45.60

<0.001*
0.726
<0.001*
1.000
0.007*
0.028*

Abbreviations:
AGE Acute gastroenteritis
CI
Condence interval
GBS Guillain-Barr syndrome
S.D. Standard deviation
URI Upper respiratory infection
* P < 0.05.

Epstein-Barr virus (13.5%), three with human herpesvirus-6

(8.1%), two with inuenza A (5.4%), and one with Varicella
zoster virus (2.7%). No pathogenic organism was identied
in the other 14 patients.

Cerebrospinal uid and neuroimaging

Cerebrospinal uid studies were performed in 34

patients. The cerebrospinal uid total protein levels ranged
from 15.5-300 mg/dL, with a leukocyte count of 0-28
cells/mm3. The mean cerebrospinal uid protein concentration was 107.32 mg/dL in 34 (79.1%, 34/43) patients. A
cerebrospinal uid mean cell count of 4.72 cells/mm3 was
detected in 25 patients, all of whom all manifested albuminocytologic dissociation on cerebrospinal uid examination (i.e., an elevated protein concentration without
pleocytosis). The cerebrospinal uid ndings were not
different between the typical Guillain-Barr syndrome and
the clinical variant group.

Spinal magnetic resonance imaging was performed in 21

patients. In the typical Guillain-Barr syndrome group,
gadolinium spinal magnetic resonance imaging (n 9)
revealed radicular root enhancement of the spinal nerve
(n 2), cauda equina (n 2), and negative ndings (n 5).
In the clinical variant group, spinal magnetic resonance
imaging indicated brainstem hyperintensity on T2-uid
attenuated inversion recovery images (n 3), and radicular
root enhancement of the cauda equina on gadolinium spinal
magnetic resonance imaging (n 2). The cerebrospinal uid
ndings, imaging, treatments, hospital stays, and outcomes
of the two groups are summarized in Table 3.
Treatment

Various therapeutic regimens used in this study included

therapeutic plasmapheresis in two (4.7%) patients, intravenous immunoglobulin only in eight (18.6%), steroids only
in 22 (51.2%), combination steroids and plasmapheresis or
intravenous immunoglobulin in eight (18.6%), and

J.-J. Lin et al. / Pediatric Neurology 47 (2012) 91e96

95

Table 3. Cerebrospinal uid ndings, images, treatment, hospital days, and functional outcomes in typical Guillain-Barr syndrome and atypical variants

CSF
WBC (n 25) (mean
S.D.)
TP (n 34) (mean
S.D.)
MRI (n 21)
Abnormal
Normal
Treatment
Supportive
Plasmapheresis
Steroid
IVIG
Combined
Hospital days (mean
S.D.)
1-year follow-up
Functional grading of disease
0
1
2
3
4
5
Outcome
Favorable (Hughes scale, 0-2)
Poor (Hughes scale, 3-5)

Typical GBS (n 29)

Atypical Variants (n 14)

4.9
8.6 (cells/mm3)

100.9
70.4 (mg/dL)

4.5
7.6 (cells/mm3)

119.1
88.8 (mg/dL)

0.914
0.515

5
7

0.623

4
5
2
2
15
5
5
14.10
7.30

1
0
7
3
3
22.71
15.79

12
12
5
0
0
0

2
4
2
5
1
0

29
0

8
6

P Value

0.07

<0.001*

Abbreviations:
CSF Cerebrospinal uid
GBS Guillain-Barr syndrome
IVIG Intravenous immunoglobulin
MRI Magnetic resonance imaging
S.D. Standard deviation
TP
Total protein
WBC Leukocytes
Functional grading of disease refers to the scale of Hughes et al. [11].
* P < 0.05.

supportive care in three (7.0%). In the clinical variant group,

steroids only were used in seven patients, intravenous
immunoglobulin only in three, combination steroids and
plasmapheresis or intravenous immunoglobulin in three,
and supportive care in one.
Outcomes at 1-year follow-up

On follow-up after 1 year or more, the functional aspects

of patients were analyzed. Follow-up study revealed that 37
patients recovered satisfactorily, and six remained with
residual paresis and an assisted gait. No mortality was
recorded during hospital admissions. The clinical variant
group, after the acute phase, contained the most children
who required extended rehabilitation. Eight patients
recovered, and six remained with residual paresis and
an assisted gait. Of the six with residual paresis, two
manifested the pharyngo-cervical-brachial variant, two
manifested Bickerstaff brainstem encephalitis, one manifested acute motor-sensory axonal neuropathy, and one
manifested acute motor axonal neuropathy. In the typical
Guillain-Barr syndrome group, all patients recovered.
Patients were divided into two groups based on functional status (favorable, Hughes score of 0-2; poor, Hughes
score of 3-5), and were compared according to their status
after 1 year. The mean disability score in the clinical variant
group was signicantly poorer than in the typical GuillainBarr syndrome group (P < 0.001). Poor outcomes were
likely attributable to respiratory signs (odds ratio, 32.00;

95% condence interval, 3.07-333.79; P 0.001), the need

for ventilator support (odds ratio, 8.25; 95% condence
interval, 1.22-55.56; P 0.045), urinary incontinence (odds
ratio, 2.20; 95% condence interval, 1.15-4.20; P < 0.001),
and etiology (clinical variant group; odds ratio, 1.75; 95%
condence interval, 1.11-2.75; P < 0.001).
We also divided the patients into a steroid only-treated
group and an intravenous immunoglobulin/plasmapheresis only-treated group, and compared the status of
outcomes at 1-year follow-up. The outcomes were not
statistically different between these two groups.

Discussion

Guillain-Barr syndrome is an acute inammatory polyneuropathy. Clinically, motor weakness and sensory loss
begin in the lower extremities, and symmetrically and
progressively ascend to the upper extremities [1-3]. The
diagnosis is relatively easy in patients with typical clinical
presentations and neurophysiologic ndings. However, as
more clinical research is focused on this disease, it has
become increasingly clear that Guillain-Barr syndrome is
not a single entity, but rather a clinically and pathologically
heterogeneous group of neuropathic conditions. A number
of Guillain-Barr syndrome variants are characterized by
localized or regional involvement of the peripheral and
autonomic nerves [4-7]. The recognition of atypical cases is
important because it permits the anticipatory monitoring of

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J.-J. Lin et al. / Pediatric Neurology 47 (2012) 91e96

complications, and identies therapeutic options for

affected children.
From a clinical standpoint, the diagnostic criteria for
Guillain-Barr syndrome do not encompass the full clinical
spectrum of this disorder, and diagnosis is further complicated when the clinical presentation is not classic. On the
other hand, a presentation that broadly ts the clinical
boundaries of Guillain-Barr syndrome may cause
a tendency to overlook other possible diagnoses, some of
which can result in death if not recognized early enough
[4,5]. In 1993, the diagnostic criteria for Guillain-Barr
syndrome were widened under the auspices of the World
Health Organization. These criteria are now exclusively
based on clinical factors [12]. As a consequence, subgroups
are proposed in a descriptive way. These new criteria do not
resolve the question of how to judge all of the different
characteristics that may occur in individual patients. A
schedule for subclassication has been devised to cover the
clinical variants in a systematic manner [8,9].
In the present study, a diagnostic owchart for GuillainBarr syndrome was developed by taking into account the
classications already suggested in the literature, and by
ensuring that the proposed classication will be able to
cover all varieties of Guillain-Barr syndrome. In the rst
step of this diagnostic owchart, patients are classied into
two groups according to their clinical presentation, as either
typical ascending form or atypical presentations. Second,
for the typical ascending group, subtypes are then classied
according to electrodiagnostic study into demyelination
forms and the axonal form. In the atypical presentation
group, subtypes are classied according to atypical clinical
presentations, and neurophysiologic ndings are classied
into prominent cranial nerve involvement and others. The
critical differences among these subtypes are mainly
observed in terms of clinical presentation and electrodiagnostic study.
In early stages, recognizing the clinical variants of Guillain-Barr syndrome can be very difcult. The main features
of Guillain-Barr syndrome include a rapidly progressive
bilateral and relatively symmetric weakness of the limbs,
with or without involvement of the respiratory muscles or
cranial nerve-innervated muscles. In typical cases, associated signs include pain, numbness, and paresthesia [8,9].
However, in clinical variants, some features may raise doubts
about a diagnosis of Guillain-Barr syndrome. Atypical
presentations include asymmetric weakness, weakness
initially involving only the arms, rapidly progressive deterioration in pulmonary function with a relative preservation
of muscle force in the extremities, and autonomic dysfunction [3]. According to the clinical experience of our study,
children with clinical variants are more likely than children
with typical Guillain-Barr syndrome to manifest rapid
progression from the onset of illness to nadir, with
increasing severity in mean disability scores and the Hughes
scale at nadir, cranial nerve involvement, and urinary
incontinence (P 0.014, P 0.004, P 0.004, P < 0.001, and
P < 0.001, respectively).
Children with Guillain-Barr syndrome present a low
risk of respiratory complications, such as respiratory
insufciency with ventilator support during hospitalization.
Only 13% of pediatric patients needed respiratory support
during hospitalization [13]. In the present study, seven

patients (16.3%) received ventilator support. In the clinical

variant group, the numbers of patients with respiratory
signs (seven of 14 children) and ventilator support (ve of
14 children) were signicantly higher than those with
typical Guillain-Barr syndrome (three of 29 children and
two of 29 children, P 0.007 and 0.028, respectively). With
respiratory support, these children demonstrated good
recovery from severe respiratory complications in the acute
stage, and all were extubated. Thus, at the onset of the
disease, rapid progress from illness onset to nadir,
increasing severity in mean disability scores and the
Hughes scale at nadir, cranial nerve involvement, urinary
incontinence, respiratory symptoms, and the need for
ventilator support may be helpful in the early recognition of
clinical variant forms of Guillain-Barr syndrome.
Steroids are not recommended in the treatment of
Guillain-Barr syndrome, and such treatment has been
rigorously studied in adults. However, immunotherapy for
children with Guillain-Barr syndrome has not been extensively studied with randomized, well-controlled studies.
Nevertheless, plasmapheresis and intravenous immunoglobulin now constitute the recommended treatments in
this age group [14].
In conclusion, during the early stage of Guillain-Barr
syndrome, increasing severity in mean disability scores and
the Hughes scale at nadir, cranial nerve involvement,
urinary incontinence, respiratory signs, and the need for
ventilator support are associated with poor prognoses. The
recognition of atypical presentations of Guillain-Barr
syndrome is important for determining appropriate treatments and improving prognoses.
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