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Pediatric Neurology
journal homepage: www.elsevier.com/locate/pnu
Original Article
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
Introduction
92
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
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.
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
Results
Demographic data
93
94
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.
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)
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.
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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