Original Article

Outcome in Childhood Guillain-Barré Syndrome

Veena Kalra, Naveen Sankhyan, Suvasini Sharma, Sheffali Gulati, Rama Choudhry1 and
Benu Dhawan1

Departments of Pediatrics and 1Microbiology, All India Institute of Medical Sciences, New Delhi, India

ABSTRACT
Objective. To assess the outcome of children diagnosed with Guillain-Barré syndrome (GBS), followed up for a median
duration of 25 months.
Methods. Tertiary center, prospective follow up of children with GBS enrolled from Dec 2003 through Sep 2006. Functional
recovery was determined at 12 months and later using Hughes scale (0-6). Clinical, electrophysiological variables were
compared between the good outcome (grade 0/1) and bad outcome groups (died or functional grade >1).
Results. Among 52 children with a median age of five yr there was male preponderance (75.4%). Mortality during acute
phase was 11.5% (6/52). Among the survivors long term data was obtainable in 40 of the 46 children. At one year follow up
87.5% children had fully recovered or had minimal symptoms, beyond one year this rose to 95%. Only 2 among 40 had
significant symptoms at last follow up (1 grade-2 and 1 grade-3). Factors significantly associated with poor outcome were:
need for artificial ventilation, inexitable nerves on nerve conduction testing and delayed independent walking.
Conclusion. Children needing ventilation have the worst short-term prognosis. However, irrespective of severity during acute
phase, good long-term recovery can be expected in most children. [Indian J Pediatr 2009; 76 (8) : 795-799] E-mail-
drnsankhyan@yahoo.co.in.

Key words: Prognosis; Outcome; GBS; Acute polyneuropathy

With the reduction of poliomyelitis, Guillain-Barré
syndrome (GBS) has become the most common cause of
acute flaccid paralysis in both the developed and
developing countries. It affects 0.6-4 individuals per
lakh population per year. 1 In the past, GBS was
considered a single disease but now it has become clear
that this clinical picture can be produced by different
pathological subtypes.2 In north America and Europe,
typical patients with GBS usually have the demyelinating
variant termed acute inflammatory demyelinating
polyneuropathy (AIDP).3 Studies from China, Japan, and
Central and South America show that axonal forms of the
syndrome [acute motor axonal neuropathy(AMAN), acute
motor sensory axonal neuropathy(AMSAN)] account for
30-47% of the cases.4 ,5 ,6 There is a paucity of comparable
data from India.
AIDP and the two axonal types usually cause
symmetric ascending flaccid paralysis, which can affect
Correspondence and Reprint request: Dr. Naveen Sankhyan,
Senior Resident, Division of Pediatric Neurology, Department of
Pediatrics, All India Institute of Medical Sciences, New Delhi,
110029, India
[DOI--10.1007/s12098--009--0125--y]
[Received June 06, 2008; Accepted October 24, 2008]
all four limbs, cranial nerves and muscles of
respiration. During the acute phase disability can be
severe and can result in respiratory in-sufficiency and
death. 7 ,8 Age is an important factor determining
outcome, and prognosis in children is said to be
favorable as compared to adults. Several retrospective
pediatric studies have tried to look at factors affecting
prognosis.9 ,10 However, studies prospectively looking
at the outcome in childhood GBS are sparse. Moreover
the regional variability in the predominant pathology of
GBS may have important implications on prognosis as
well. So, the present study was undertaken with a view
to prospectively evaluate the clinical profile, short term
and long term follow up of childhood GBS in Indian
population.
MATERIAL AND METHODS
This is a prospectively conducted study at a single
tertiary care research center of North India. Over a
period of three years (December 2003 to September
2006), all children with GBS aged <16 years were
enrolled for the study. Ethical clearance was obtained
from the institutional ethics committee. Informed
consent was obtained from all parents before
enrollment. Many patients were actively sought as part

Indian Journal of Pediatrics, Volume 76—August, 2009 795

 Veena Kalra et al
of a synchronous study investigating the relation
between previous Campylobacter jejuni infection and
Guillain-Barré syndrome. 11 Diagnosis of GBS was
accepted after children were examined at least once by
a consultant pediatric neurologist and the case fulfilled
the criteria of Asbury and Cornblath. 12 All patients
diagnosed as GBS were admitted. IVIG therapy (2g/Kg
over 2-5 days) was given in those unable to walk or had
evidence of respiratory muscle weakness. 13 Till the
course was static or improving and there were no
potentially life threatening features the child remained
admitted.
Nerve conduction studies could be done in 33
(63.4%) of the enrolled children. Motor nerve
conduction, sensory nerve conduction and F wave
response studies were performed by using the standard
technique of supramaximal percutaneous stimulation
and surface electrode recording. Motor conduction was
studied in median, ulnar, tibial and common peroneal
nerves. Sensory conduction was studied in median,
ulnar and sural nerves.
Lumbar puncture was done at presentation or in the
second week of illness in those presenting early. All
children underwent stool poliovirus detection as per
the national policy. For maintaining uniformity,
outcome was assessed at one year after onset of illness
on the Likert scale. 14 In this scale; zero indicates-
Healthy: no signs or symptoms due to Guillain-Barré
syndrome, 1- minor symptoms or signs and capable of
running, 2-able to walk 5 m across an open space
without assistance, walking frame, or stick but unable
to run, 3-able to walk 5 m across an open space with the
help of one person and waist level walking frame or
sticks,4 chairbound or bedbound: unable to walk as in
3, 5-requiring assisted ventilation for at least part of
day or night, 6-dead.
For children with any disability at one year,
outcome was assessed till last contact. Follow up was
assessed on outdoor follow up visits and also by
telephonic interviews and by postal communication in
all patients. At least three attempts (2 postal reminders)
were made to contact all non responders. Out of a total
of 52 children enrolled, 46 were alive at discharge and
one year follow up was available in 40.
The SPSS-15 statistical program was used for
analysis. We compared the clinical, laboratory and
electrophysiological features among children with
good outcome (score 0 or 1) and those with poor
outcome (score 2-6). 15 Due to non-parametric
distribution of data, the Mann Whitney U test was used
for continuous variables and Fischer’s exact test for
categorical variables. We evaluated our findings
against a two-tailed significance level of 5% (p<0.05).
The paucity of children with poor outcome prevented
us from identifying independent predictors using a
regression model.
796
RESULTS
Between Dec 2003 and Sep 2006, 52 children with a
diagnosis of GBS were admitted to our center. The age
range was 12 months to 15 years, median age being
five years. There was a striking male preponderance,
the female: male ratio being 1:3 (girls-13, boys-39).
Mortality during acute phase was 11.5%. Five of these
six children had respiratory failure; one each had
respiratory failure with encephalopathy and bulbar
paralysis with aspiration pneumonitis. A preceding
infective illness was reported in 30(57.7%) of children.
Eleven had an upper respiratory infection, eight had
diarrheal episode and eleven had a non-specific febrile
illness. Thirty one (59.6%) children had myalgias or
paraestheisas at the onset. Weakness was present in all
children at presentation. The median duration to peak
weakness was seven days (range-1-24 days), and
majority (69.2%) had upper limb involvement in
addition to lower limb weakness. Bulbar weakness was
evident in 20(38.5%) and other cranial nerve
involvement was seen in 10 (19.2%). Dysautonomia
was detected in 9(17.3%). Respiratory muscle weakness
was seen in 15(28.8%) out of which 10(19.2%) received
artificial ventilation. IVIG therapy was given to
43(82.7%) children. Nerve conduction studies were
done in 33 children, 18(54.5%) had reduced compound
muscle action potential amplitudes with normal
velocities indicative of axonal dysfunction or distal
demyelination, 5(15.1%) had reduced velocities
indicating diffuse demyelination and 10(30.3%) had
inexicitable nerves.
Long-term data was obtainable in 40 out of 46
children discharged after the acute phase of illness. The
median follow up duration was 25 mth (Range:12-48
mth). At one year follow up 87.5% of them (grade 0-28,
grade1-7) either recovered fully or had only minimal
symptoms. Among ambulatory children the median
time to unaided ambulation was 42 days (5-360days).
Among 15 children with respiratory muscle
involvement (10 - ventilated), 5 died in acute phase.
Among eight with follow up data, seven had good
recovery (grade <2) at one year and the lone child with
functional grade 2 had recovered to grade 1 at 22 mth.
Factors significantly associated with poor outcome
(grade 2-6) at one year follow up were; artificial
ventilation, inexitable nerves on nerve conduction and
delayed independent walking (Table 1). Of the 7 with
grade1 disability 2 were symptomless at 24 and 30
months follow up, rest of the five had static disability
(Fig. 1). All 3 children with grade 2 disability had
improved to grade 1 on follow up. One child with grade
3 disability had improved to grade 2 by 36 mth. One
child among those enrolled had a history of previous
GBS and among those followed up, no case of recurrent
GBS was seen.
Indian Journal of Pediatrics, Volume 76—August, 2009
 Outcome in Childhood Guillain-Barré Syndrome

DISCUSSION

GBS was described more than a century back but the

data on prospective long term follow up of childhood
GBS is still sparse. Reports have suggested differences
in clinical presentation and course of this illness among
children as compared to adults. 7,8,9,14,15 Hence it is
imperative that data from pediatric cohorts is separately
evaluated. We bring results of the largest prospectively
collected data on outcome of GBS in Indian children.
Males are reported to be around one and half times
more likely to develop GBS compared to females.2 But we
believe the striking male preponderance in our series is
in part related to the extra attention and concern shown
to the male child in this region of our country, accounting
for higher health facility visits for their illnesses. In a
prospective study of 95 children with GBS, 73.7% had a
recent infection.16 Fifty seven percent of the children in
the present study had a recent infection, upper Fig. 1. Flow of Children with GBS during the follow up period.
respiratory infections being the commonest. *Duration of Follow up in months
At the nadir of the illness, the disease is less severe in
TABLE 2. Prognosis of GBS in Different Studies
children than adults, 30-40% are able to walk,8,15 13-20%
require artificial ventilation and 50% have Authors Age range Number Ventilated Mortality Recovery
dysautonomia. 7,15 Respiratory paralysis, secondary (%) (%) (%)
infections, bulbar weakness, multisystem involvement Paradiso et al5 14 mo-14 yr 61 13.1* NA 82% (14-
and dysautonomia can be life threatening during the 270 days)
acute phase. 8,15,17 Rates of mortality have varied in Korinthenberg
et al 7 11 mo-17.7 yr 155 16 NA 92.4%
different studies depending partly on the population (≥6 mo)
Rantala et al 9 0.4-14.3 yrs 27 18.5 0 NA
Briscoe et al 10 19 mo- 13 yr 23 4.3 4 NA
TABLE 1. Comparison of Children with Good and Poor Kleyweg et al 25
1-14 yrs 18 22 11.1 77% (Gr0/
Outcome 1 at 1 year)
Rees et al 15 5-85 yrs 69 25 8.7 NA
Variable Good recovery Poor recovery P value Beghi et al8 3-87 yrs 297 14 11 70%
( 1 year) Present
(Gr <2) ‡ (Gr 2-6) ‡ study 1-15 yrs 52 19.2 11.5 87.5 %(Gr
(n=35) (n=11) 0/1 at 1 yr)
Age (years) 5.6 ± 3.2 6.0 ± 4.2 0.92
*Data on respiratory failure, NA- Not available
Male Gender 27 (77.1) 8(72.7) 1.0
Preceding infections† 22(62.9) 6(54.5) 0.72
Quadraparesis 23(64.5) 10(90.9) 0.14 studied, therapy and facilities (Table 2). In our series
Bulbar weakness 12(34.3) 6(54.5) 0.29 the high rate of ventilation (19.2%) and acute phase
Cranial neuropathy 7(20) 3(27.3) 0.68
mortality(11.5%) reflect in part the severer end of the
Respiratory muscle
involvement 8 (22.9) 6(54.5) 0.06 disease spectrum seen at our center. Meticulous
Dysautonomia 7(20) 3(27.3) 0.68 respiratory care and monitoring and recognition of
Bladder involvement 1(2.9) 2(18.2) 0.14 bulbar weakness and dysautonomia could possibly
Onset to peak (days) 8.8 ± 5.8 10.7±6.2 0.33 reduce the mortality further.
CSF†† proteins (mg/dl) 77.4 ± 46.1 95.8±49.8 0.33 Immunotherapy has long been the mainstay of
Nerve conduction studies
Reduced velocities 3(13) 1(14.3) 1.00 therapy of GBS apart from supportive care. Due to the
Reduced ease of administration and lower cost,
amplitudes 15(65.2) 0(0) 0.006* immunoglobulins are commonly used to treat GBS,
In excitable nerves 5(21.7) 5(71.4) 0.026* especially in those patients who are unable to walk. 13
IVIG therapy 28(80.0) 9(81.8) 0.89 We used IVIG; however its effect on outcome cannot be
Artificial ventilation 3(8.6) 5(45.5) 0.013*
Time to walk
assessed in an uncontrolled trial like the present study.
independently (days) 61.0±53.9 168.7±139.3 0.026* Artificial ventilation was associated with poor
outcome in the present study. Need for ventilation
Number in parenthesis indicates percentage, indicates severity at the nadir, a variable found by most
†–preceding infections- include gastroenteritis, upper researchers to have a bearing on outcome. 7,8,14,15,18 ,19
respiratory infections and non specific febrile illnesses
Poorer outcome has also been related to low amplitude
††–data available for 42 of 46 patients analysed
‡Grades (0-6) as described in methods 14 responses on nerve conduction,18 axonal degeneration 8

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 Veena Kalra et al
or electrical non excitability.18,19 Previous reports and
our study, suggests that the finding of low amplitudes
on nerve conductions may not necessary imply a bad
outcome. 20 ,21 Two patterns of recovery are found in
patients with AMAN; rapidly recovering conduction
block at the nodes of Ranvier and more protracted
recovery in those with extensive axonal degeneration.22
In the present study too, two distinct patterns emerged,
a large majority with low CMAP’s and quick recovery
and another group with inexicitable nerves and poor
recovery. The finding of inexicitablity of nerves was an
indicator of poor outcome, in this and previous
reports.19,23 Inexicitablity can result either from axonal
degeneration or dysfunction or demyelination causing
complete conduction block.24 Other factors predicting
poor outcome include, a protracted plateau time,
change to chronic progressive or relapsing
neuropathy10,16 or concurrent myelitis.16 A longer time to
independent walking was an indicator of poorer long
term recovery in the present series.
Long term recovery in most pediatric series has been
better compared to adults (Table 2). In the largest
pediatric prospective study by Korinthenberg et al, at the
end of the observation period (288 days), 96% of children
were either asymptomatic or had symptoms not affecting
daily functioning. 16 We also found the outcome to be
excellent with full recovery or minimal symptoms in
87.5% children at one year. Many of those with weakness
at one year continued to improve beyond one year too,
with 95% making good recovery at last follow up (Fig 1).
This late recovery has been observed by other researches
too.23 The favorable long term outcome in children calls
for further large studies looking at early predictors of
severity and mortality and effects of combinations,
sequential administration and repeat courses of existing
immunotherapies.
CONCLUSION
Excellent recovery can be expected in nearly all
children with GBS, provided respiratory, bulbar and
autonomic impairments during the acute phase are
managed meticulously. While most children recover
within six months, continued improvement occurs
beyond one year.
Contributions: The concept and design of the study was
provided by VK, RC and BD. Data was collected, analyzed by
NS, S Gan. NS, SS reviewed the literature and drafted the
manuscript for which SG provided important inputs. The
manuscript was finally revised and approved by VK,RC and BD.
Conflict of Interest: None
Role of Funding Source: This study was supported by the
Department of Biotechnology, Govt. of India through financial
grant reference D.O.No.BT/PR2193/Med/09/322/2000.
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