Guillain Barre Syndrome : Major Cause of Acute Flaccid Paralysis in Children and Adolescents of Nepal

Introduction. Guillain Barre Syndrome (GBS) is a post infectious polyneuropathy involving mainly motor but sometimes sensory and autonomic nerves. It is an acquired disease of the peripheral nerves that is characterized by rapidly progressing paralysis, areflexia and albumino-cytological dissociation in CSF. Methodology: Prospective, descriptive, observational, hospital based study was carried out to find out the clinico-epidemiological features of GBS including existing treatment modalities and its outcome. All cases fulfilled the criteria for AFP (Acute flaccid Paralysis) surveillance was included. Cases were reviewed for full medical history and examinations. To confirm the diagnosis, necessary investigations were carried out and combined with clinical symptoms. Results: Thirty patients were included in the study during study period. Among them 90% were diagnosed as GBS, 7.4% patients of GBS were associated with hypokalemic paralysis, 7.4% diagnosed as transverse myelitis and 3.7% diagnosed as idiopathic neuropathy. Different types of GBS were classified as AIDP (Acute inflammatory demyelinating polyneuropathy) 62.96%, AMAN (Acute motor axonal neuropathy) 25.52%, AMASAN (Acute motor and sensory axonal neuropathy) 3.3% and MFS (Miller fisher’s syndrome) 6.6% according to NCV result. Male female ratio is 1.7:1.0. There was 14.8% patients had relapse within 5 year. Associated diseases were URTI, pneumonia, sore throat and diarrhea. Facial Nerve palsy was commonest cranial nerve involvement.Sixty percentage of patients presented with sensory symptoms. There was transient bowel and bladder involvement in 20% of the cases. 69.2% patients became bed ridden at the nadir. There was albumin-cytological dissociation in 80% case. Majority of patients improved with supportive treatment alone, 19.5% patient required ventilator support among them 40% died. 7.4% of cases expired during treatment. Half of the patients fully recovered within 3 months. Conclusion: GBS is the commonest cause of AFP, AIDP being commonest subtype in our setting. We have to improve our existing treatment facilities and extend to different centers to detect and treat GBS. Most of the patients improve with supportive treatment alone. Ventilator support indicates grave prognosis.


Introduction
G uillain Barre Syndrome (GBS) is a post infectious polyneuropathy involving mainly motor but sometimes also sensory and autonomic nerves 1 .GBS is an acquired disease of the peripheral nerves that is characterized clinically by rapidly progressing paralysis, arefl exia and albumino-cytological dissociation 2 .Earliest description of this disease is probably that of Word Rob and Oliver in 1834, but the syndrome as fi rst described in 1859 by Jean Baptiste Octave Landry, who identifi ed the clinical features of the disease and termed it as 'Acute ascending paralysis.Osler reported the same disease in 1982 as febrile polyneuritis 3 .In 1916, Guillan, Barre and Strohl published their observation of the albumin cytological dissociation on cerebrospinal fl uid examination of two shoulders who had presented with paralysis and arefl exia 2 .A comprehensive pathological account of GBS was that of Haymaker & Kernohan  (1949) who stressed that edema of the peripheral nerves was the important changes in the early stage of disease 3 .Asbury, Arnoson and Adam (1969) established the essential lesion; it is a perivascular mononuclear infl ammatory infi ltrate of the root and nerves 3 .In 1980s and 1990s saw the development of plasma exchanges, intravenous immunoglobulin and all of which reduced the mortality and morbidity 2 .The watershed development in the last decade, however, has been the characterization of variants of GBS and recognition of pathological association of campylobacter jejuni infection with the motor axonal form of GBS 3 .
GBS occurs in all age groups and has an annual incidence varying from 0.4 to 1.7 cases per 100000 population.The reported incidence for children under 15 years is similar to adults 2 .It occurs in all part of the world in all seasons, affects both children and adult of both sex 2 .In post polio era, it is the most common cause of an acute generalized paralysis 2 .Though GBS is nonseasonal and non-epidemic, the existence of an axonal variant of GBS has been confi rmed by observations on an annual seasonal epidemic that occurs in northern China in summer 4 .There is a slightly male predominance with male to female ratio of 1.5:1.whilemost of the cases occurs sporadically; occurrence of epidemic clusters of GBS has been described 2

Materials and Methods
Prospective, descriptive, observational, hospital based study was carried out at to fi nd out the clinicoepidemiological features of GBS in eastern part of Nepal including clinical presentations, risk factors, existing treatment modalities, prognosis of the disease in relation to its severity and outcome in the tertiary care centre.All cases fulfi lling the criteria for AFP surveillance were included in study.Children enrolled in study were seen in emergency, outpatient department (OPD), Pediatric wards and pediatric intensive care unit (PICU).Cases were reviewed for full medical history, general physical and detail systemic examination, in order to confi rm the diagnosis, necessary investigations were made.All children up to 15 years, coming under AFP surveillance were included.Features required for Diagnosis includes progressive weakness of both legs and arms and arefl exia 7 .Clinical features supportive of diagnosis includes progression over days to a couple of weeks, relative symmetry of signs, mild sensory symptoms or signs, cranial nerves involvement (bifacial palsies) Recovery beginning 2-4 wks after progression ceases, autonomic dysfunction, and absence of fever at onset 7 .Laboratory features supportive of diagnosis includes elevated CSF protein with < 10 cells/micro liter and electro diagnostic features of nerve conduction slowing or block 7 .

Statistical Analysis
The data collected were compiled and entered in MS Excel.Statistical analysis was carried by using SPSS pc+ 11.0 versions to fi nd out different type of correlation and statistical values.P value < 0.05 was taken as a statistically signifi cance.

Results
Thirty patients were included in the study during 16 months of study period.Among them 27 (90%) were diagnosed as GBS, 2 (7.4%) patients of GBS were associated with hypokalemic paralysis, 2 patients (7.4%) diagnosed as transverse myelitis and 1 patient (3.7%) diagnosed as idiopathic neuropathy.
GBS is commonest cause of AFP, AIDP being commonest subtype in our setting.There was associated preceding illness in GBS.Majority patients presented with symmetrically ascending paralysis with gradual onset involving all limbs.Male were more affected in present study with male female ratio as 1.7:1.0.There was no signifi cant age distribution but slightly higher patient from pre adolescent to young adults were found.There was no seasonal variation, no signifi cant status with immunization, but 14.8% patients had relapse within 5 year.Associated diseases were URTI, pneumonia, sore throat, diarrhea in majority of patients.
Facial Nerve palsy was commonest cranial nerve (CN) involvement, 60% of patients presented with sensory symptoms like pain, tingling and numbness.There was transient bowel and bladder involvement in 20% of the cases.All patients became arefl exic during the course of illness with 69.2% becoming bed ridden at the nadir.77.8% patients had elevated CSF protein and 88.2% patients had less than 10 CSF lymphocytes after 2 week of illness.63% patients had acute infl ammatory demylinating polyradiculopathy.23 patients required only supporting treatment including steroids and physiotherapy but fi ve patients required ventilator support.Two patients were expired during treatment (On Ventilator due to respiratory failure).63% of patient discharged within 1 week of admission.Hospital mortality was 7.4% and 85.18% were discharged after improvement.Out of two one patient expired after 32 days due to secondary infection with aspiration, next patient expired immediately after admission on day 1 due to autonomic instability.Hughes scale was used for monitoring of weakness,43.47%patients became healthy at 3 month of illness; other patients were improving only 8.69% patients remain bed ridden without further deteriorations.

Nerve Conduction Velocity Study
There was no signifi cant correlation found between different modes of clinical presentation like duration of weakness, type of weakness.Involvement of cranial nerve was analyzed with SPSS pc+ 11.0 version and no signifi cant correlation found.There was albumincytological dissociation in 80% case.Majority of patients improved with supportive treatment alone, 19.5% patient required ventilator support among them 40% died.Majority of patient discharged within 1 week of admission with some improvement.7.4% of cases expired during treatment, at 3 month follow-up almost half patients recovered fully and remaining patients improved on follow up.

Discussion
GBS is commonest cause of AFP, AIDP being commonest subtype in our setting.There was associated preceding illness in GBS.Majority patients presented with symmetrically ascending paralysis with gradual onset involving all limbs.Male were more affected in present study with male female ratio as 1.7:1.0.There was no signifi cant age distribution but slightly higher patient from pre adolescent to young adults were found.There was no seasonal variation, no signifi cant status with immunization, but 14.8% patients had relapse within 5 year.Patient had associated illness like URTI, pneumonia, sore throat, diarrhea in majority patients.Facial Nerve palsy was commonest cranial nerve involvement, in 60% of patients presented with sensory symptoms like pain, tingling and numbness.There was transient Bowel and bladder involvement in 1/5 th of the cases.All patient became arefl exia during the course of illness with 69.2% became bed ridden at nadir.There was albumino-cytological dissociation in 80% case.Majority patient improved with supportive treatment alone, 19.5% patient required ventilator support among them 40% mortality occurred.Majority of patient discharged within 1 week of admission with some improvement.7.4% cases are expired during treatment, at 3 month follow-up almost half patient become healthy and remaining patient were improving on follow up.GBS is the commonest cause of the acute fl accid paralysis.It has been observed in Nepal from long time, which was recorded nationwide through AFP surveillance.Our present study was carried out to fi nd out the clinico-epidemiological features of GBS in eastern part of Nepal (carried out at BPKIHS), the only tertiary care centre in Eastern Nepal.

Conclusion
GBS is the commonest cause of AFP, AIDP being commonest subtype in our setting.We have to improve our existing treatment facilities and extend to different centers to detect and treat GBS.Most of the patients improve with supportive treatment alone.Ventilator support indicates grave prognosis.Adequate supportive treatment and long term follow up is needed to insure recovery from illness.Over all cures is good except respiratory failure.