Clinical Profile and Outcome of Guillain–Barre Syndrome in Pediatric Patients Admitted to a Tertiary Care Centre: A Retrospective Study
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0028-3886.310112
Source of Support: None, Conflict of Interest: None
Keywords: Acute motor axonal neuropathy, autonomic disturbances, demyelination, Guillain Barre syndrome, MRI spine, neuropathy, paralysis
Guillain Barre Syndrome (GBS) is an autoimmune disorder with post-infectious polyneuropathy involving motor, sensory and sometimes the autonomic nerves. With the eradication of wild polio virus infection, GBS has become the most common cause of acute flaccid paralysis in both the developed and developing countries. The world-wide incidence of this disease is about 1.3 per lac per year. GBS is characterized by ascending paralysis (lower limb numbness, paraesthesia or pain followed by weakness which ascends symmetrically and gradually progresses over a period of 1 to 28 days with maximum severity of weakness by four weeks after the onset). Bulbar involvement is seen in 50% cases and autonomic disturbances are seen in about 20%. Since there may be mortality related to acute complications of respiratory muscle paralysis and morbidity related to the long duration of the paralysis, we retrospectively analysed the clinical presentation, antecedent illness, treatment given, need for mechanical ventilation, the complications, outcome and the mortality in patients diagnosed as GBS.
This was a retrospective descriptive study conducted in the pediatric wards and pediatric intensive care unit (PICU) of a multispeciality (teaching and referral) tertiary care hospital from a metropolitan city of Western Maharashtra (India). The study enrolled patients over a period of two years (from August 2016 to July 2018). The study was initiated after approval from the Institutional Ethics Committee of our institution. All patients who were clinically diagnosed as GBS (Guillain–Barre Syndrome), that is, those presenting with flaccid paraparesis/quadriparesis/paraplegia/quadriplegia, depressed/absent deep tendon reflexes, absent plantar reflexes, and cranial nerve involvement (lower motor neuron type) in the wards and intensive care unit (ICU) of the pediatric department were included. The age group ranged from 1 month to 12 years. The indoor case papers/records were used for recording the details, including the investigations and disease characteristics/progression. Patients who had an alternative diagnosis (other than Guillain–Barre syndrome, like transverse myelitis and traumatic neuritis) were excluded from the study.
The clinical details of patients diagnosed as GBS (as mentioned in the indoor case papers) were recorded in the Case Record Form designed for the study purpose. The information recorded included the demographic details (age, sex, and duration of stay), clinical features, investigations, treatment given (intravenous immunoglobulin – IVIG or plasmapheresis or steroids), complications, need for PICU admission, details of mechanical ventilation (if applicable), and the outcome (recovered—complete/incomplete or death). The criteria for PICU admission for GBS patients in our hospital included - hypoventilation or difficult to maintain airway, presence of autonomic disturbances and hemodynamic instability. The criteria for discharge of patients from our ward included adequate respiratory drive without hypoventilation or respiratory distress, hemodynamically stable patient, and improvement in clinical features (like improved diaphragmatic contractility, presence of gag reflex, improvement in muscle power, and absence of autonomic disturbances). The outcome was assessed in terms of the duration of stay, need for PICU admission, improvement/no improvement, whether discharged, and death. Descriptive statistics like mean, median, mode, and standard deviation were used for summarizing the nominal data like age, days of stay in wards/PICU, and duration of symptoms. Fischer exact test (P value <0.05 taken as statistically significant) was used to study the association between the mortality and presence of autonomic disturbances.
A total of 30 patients were included in the study of which 21 were males (70%) and nine were females (30%). The mean age of the patients was 5.4 years (median 4.5, mode 4 years, range 10 months– 11 years). The duration of hospital stay ranged from 1 day to 60 days with a mean of 13 days and SD of 14.6 days. Six patients had prolonged stay ranging from 14 to 60 days. PICU admission was required for 16 patients (53%) and the duration of PICU stay ranged from 2 to 56 days with a mean ± SD of 13.2 ± 7.9 days. Seven patients required mechanical ventilation and the duration of ventilation ranged from 3 to 51 days (mean ± SD of 16.8 ± 11.9 days, median 9, mode 3). Only one patient required tracheostomy. There were two patients who had recurrent GBS (for one it was the second episode and third episode for the other) and the rest 28 cases suffered from GBS for the first time. Presenting complaints were symmetrical lower limb weakness (26 cases; 86.67%) followed by respiratory complaints (six cases; 20%), quadriparesis (four cases; 13.33%), and facial palsy (two cases; 10%). There was no antecedent illness in nine patients, another nine had a fever, gastrointestinal complaints were seen in six cases and respiratory complaints were noted in three cases. Two patients had varicella infection (in preceding one week) and one had mumps infection (one month prior to presentation).
MRI-spine was done in 12 patients, of whom, nine patients had features suggestive of GBS (thickening and contrast enhancement of the intrathecal and cauda equina nerve roots on post-gadolinium contrast T1 weighted MRI images).Nerve conduction studies (NCV) were done in 16 patients, of which, 15 patients had features suggestive of GBS and it was normal in one patient [Table 1]. Intravenous immunoglobulin (IVIG) [total dose- 2 g/kg] was given to 27 (90%) patients. Two patients required a second course of IVIG (given 2 weeks after the first course and both patients improved clinically and were discharged). Five cases received intravenous (IV) Methylprednisolone (MPS). Three received both IVIG and MPS. The details of the clinical profile and outcome of mechanically ventilated patients is given in [Table 2]. Common complications included pneumonia [including two cases with aspiration pneumonia and 4 with ventilator associated pneumonia (total 6 cases)] and autonomic disturbances in the form of blood pressure and heart rate fluctuations in 6 cases [Table 3]. Twenty eight patients improved clinically and were discharged (93.33%) and 2 cases died (6.6%) due to autonomic disturbances. Autonomic disturbances predicted higher mortality (P = 0.034).
The present retrospective study was done to analyze the clinical profile and outcome of Guillain–Barre syndrome in pediatric patients. Nerve conduction studies were done in 16 patients in our study, of whom acute motor axonal neuropathy (AMAN) was the most common finding and all these patients improved clinically and were discharged. This was similar to the study by Kalra et al. who also showed that AMAN may not always indicate a bad prognosis compared with AIDP (Acute Inflammatory Demyelinating Polyneuropathy) and that there are two patterns of recovery seen in patients with AMAN—firstly the rapidly recovering conduction block at the nodes of Ranvier having low CMAP (compound muscle action potential) with a good prognosis and secondly the inexicitable nerves with more protracted recovery in those with extensive axonal degeneration having a poorer prognosis. Kannan et al. also showed that AMAN subtype accounted for a significant proportion of pediatric GBS in their study and the recovery in children was complete, irrespective of the subtype. Alexander et al. compared the electrophysiological findings among adults and children and showed that AMAN is more common in children than adults. Whereas the study by Kalita et al. showed AIDP to be a more common finding and was associated with a good prognosis compared to the axonal type, it is characterized by better recovery at three months than AMAN. Meshram et al. also studied 52 children and NCV was done in only 15 patients of whom 73% had AIDP as their finding and all of them recovered completely.
Recurrent GBS in pediatric patients has not been studied extensively in India. Recurrent GBS was seen in two of our patients (6.6%);for one patient, it was the second episode for whom no antecedent illness was noticed (the child clinically improved and was discharged) and for the other it was the third episode following a varicella infection one week prior (this patient died due to autonomic disturbances and acute kidney injury). As per NINCDS definition of recurrent GBS, a minimum time between episodes of two months (when fully recovered in between) or four months (when only partially recovered) is taken and both our patients fulfilled the above criteria. Van der Pijl et al. also showed that 6% cases in their study had recurrent GBS and the second episode was more likely to be severe than the first one. Kuitwaard et al. described the risk factors for recurrent GBS being patients' age under 30 years, those with milder symptoms and with Miller Fischer syndrome. In a study by Das A, et al. in Lucknow, six pediatric patients were studied and they have stated that there seems to be a tendency to accumulate neurological deficits with increasing frequency of attacks of GBS. In a case report by Kadam S, et al., a 9-year-old girl has been reported with a second episode of recurrence who had residual, but progressively improving diplopia that had improved drastically after giving IVIG.
IVIG injection is usually burdensome for poor patients as regards to the cost of the injection; however, IVIG therapy was given to 90% of our patients within 24 to 48 hours of admission of whom 92.5% patients improved clinically and were discharged and plasma exchange was not done for any of the patients. Hence, the efficacy of plasma exchange and IVIG therapy could not be compared by us; whereas in another study conducted by Kalita et al., only 45% patients received IVIG (due to nonaffordability of the patients); however, they compared the two groups of whom IVIG was given and IVIG not given and found no significant difference in the outcome among them. Incecik et al. studied two groups of patients (one receiving IVIG and the other group receiving plasma exchange) and found no difference in the efficacy of both these therapies. Bagul et al. studied 28 patients of whom IVIG was given to only 35.71% patients who presented with acute rapid progression of weakness and cranial nerve involvement. Koul et al. showed in their study that IVIG was given to all the patients (52) and it reduced the duration of the disease, disability, hospital stay, mortality, and ventilation requirement.
In our study, 23% GBS patients required mechanical ventilation, which was similar to the study by Meshram et al. who showed that 26% patients required ventilation as against study by Nasiri et al. who showed only 8.7% patients needing mechanical ventilation. The most common complication in our study was pneumonia (20%) and autonomic disturbances (20%). The study by Kalita et al. showed the incidence of autonomic disturbances being similar (25.7%) to ours; these autonomic disturbances were lowest in a study by Nasiri et al., and highest in the study by Incecik et al. (45%).
The mortality in our study was 6.6% whereas the mortality rate in study by Kalra et al. was as high as 11% and that by Nasiri et al. was the lowest at 1.8%. Autonomic disturbances predicted a higher mortality rate (P < 0.05) in our study, similar to the study by Van der Pijl et al. who showed that autonomic instability had a significant association with poor outcome of patients.
The presenting features and investigations in our study are compared with earlier studies in [Table 4].
Limitations of the present study included- the retrospective nature of the data collection which did not allow us to characterize the disease in more details, absence of long-term follow up (for functional recovery and independent walking ability) and a small sample size (limiting the generalizability of the results).
We conclude that gastrointestinal illness was the commonest antecedent illness for GBS and symmetrical lower limb weakness was the commonest complaint. MRI spine proved to be useful (whenever available). Pneumonia and autonomic disturbances were the commonest complications while autonomic disturbances predicted higher mortality in our study.
The authors thank Dr. Hemant Deshmukh- Dean, Seth G.S. Medical College and KEM Hospital for granting permission to submit this manuscript for publication.
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Conflicts of interest
There are no conflicts of interest.
Note on authorship: Dr. Ramya Srinivasa Rangan and Dr. Milind S. Tullu are both designated as “First Authors” of this manuscript owing to their equal contribution.
[Table 1], [Table 2], [Table 3], [Table 4]