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

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Veena Kalra et al

of a synchronous study investigating the relation RESULTS


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

796 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 features of Guillain-Barré syndrome. J Infect Dis 1997; 176:
our study, suggests that the finding of low amplitudes S92–S98
2 Hughes RAC, Cornblath DR. Guillain-Barré syndrome.
on nerve conductions may not necessary imply a bad
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patients with AMAN; rapidly recovering conduction Electrophysiological classification of Guillain-Barré
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paralysis in China. Ann Neurol 1993; 33: 333–342
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ganglioside antibodies and Campylobacter jejuni infection in
poor outcome include, a protracted plateau time, Japan. Ann Neurol 2000; 48: 624-631.
change to chronic progressive or relapsing 7 Korinthenberg R, Mönting JS. Natural history and treatment
neuropathy10,16 or concurrent myelitis.16 A longer time to effects in Guillain-Barré syndrome: a multicentre study.
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