Guillain-Barré syndrome: Clinical profile and Consensus to revise Hughes grade 5

Background: Guillain–Barré Syndrome (GBS) is an acute, immune-mediated polyradiculoneuropathy and an important cause of acute flaccid paralysis (AFP) worldwide. Respiratory insufficiency requiring ventilator occurs in 30% of patients that prolong the hospital stay, leading to morbidity and mortality. There had been relatively few studies of Guillain-Barre syndrome in adults from South India. Aim: To evaluate clinical profile, epidemiological, laboratory, and electro diagnostic features of patients with GBS in adults. Settings and design: A prospective study was conducted over a period of 4 years at ESIC Superspeciality Hospital, Hyderabad. Materials and methods: Total 36 patients were identified and data was collected. We studied the epidemiological, clinical, electrophysiological features and their outcome. We subdivided Hughes grade 5 into 5A and 5B based on the requirement of ventilator. Statistical Analysis: Data obtained in the study were subjected to statistical analysis with Statistical Package for Social Sciences (SPSS) version 18. Bivariate analysis was done using chi-square test. Results: Of 36 GBS patients, 21(58.3%) were males; the mean age was 35 years. Antecedent infection was found in 23(63.8%). Majority 12 (33%) were in Hughes grade 4, 10 (27.7%) were in Hughes grade 5A. 97.2% had limb weakness. A significant association was found between low Medical research sum score (MRC) and respiratory failure. Most predominant neurophysiological variant was acute inflammatory demyelinating polyradiculoneuropathy (AIDP) 12 (33.3%). Duration of illness was less than 1week in 19 (52.7%) of cases. Asymmetry was observed in 5 (13.8%) and recurrence of Guillain-Barre Syndrome seen in 2 (5.5%) cases. Complete recovery was noted at 6 months in 34 (94.4%) cases. Conclusion: Early diagnosis of respiratory failure and prompt intervention improves patient outcome. Further large sample studies are required to assess respiratory failure and subdivision of Hughes grade 5.


INTRODUCTION
GBS is the major cause of acute neuromuscular paralysis with an annual incidence of 0.6-2.4 per 100,000 worldwide. 1,2There are no incidence studies of GBS in Indian population. 3GBS incidence increased by 20% for every 10-year increase in age; the risk of GBS was higher for males than females. 4BS can cause life-threatening complications if the respiratory muscles are affected or if autonomic nervous system is involved.The weakness reaches its nadir in 2-4 weeks.About 5% of the patients die and more patients are left with a disabling motor deficit and/ or fatigue.About 30% of patients have respiratory failure due to progressive weakness of respiratory muscles requiring ventilator. 5Aspiration pneumonia and atelectasis are common consequences of bulbar muscle weakness.Several factors at admission and during the ICU stay are known to predict a need for invasive mechanical ventilation.These include rapidly progressive motor weakness, involving the peripheral limbs and the axial muscles, ineffective cough, bulbar muscle weakness, and a rapid decrease in vital capacity.Recognition of early respiratory fail-

MATERIALS AND METHODS
A prospective study was conducted from January 2012 to January 2016 among the patients admitted in department of Neurology, Hyderabad in South India, where 36 consecutive GBS patients, underwent detailed clinical and electrophysiological assessment.Institutional Ethics Committee approved the study protocol and written informed consent was taken from all relatives of participant.Information regarding the relevant variables in the study was collected with the help of structured proforma comprising age, sex, antecedent infections, duration of illness, seasonal trend, clinical variant, recurrence, asymmetry, CSF analysis, electrophysiological variant, Hughes disability score, treatment opted and outcome assessed at 1,3,6 and 12 months.The diagnosis of GBS was made using the Asbury and Cornblath criteria. 6Medical Research Council (MRC) sum score was used for valuing the muscle strength from 0 to 5 in proxi-mal and distal muscles in upper and lower limbs bilaterally; score ranged from 40 (normal) to 0 (quadriplegic) and by Hughes et al. disability score for GBS. 7,8 e further subdivided Hughes grade 5 to 5A and 5B based on requirement of ventilator.(Table 1).Classification of patients as axonal or demyelinating subtype was based on electro diagnostic criteria of Hadden et al. 9 Data obtained in the study were subjected to statistical analysis with Statistical Package for Social Sciences (SPSS) version 18. Categorical variables were summarized as counts (percentage) and continuous variables as means or medians (interquartile ranges (IQR)).Bivariate analysis was done using chi-square test to compare between respiratory failure group and non-respiratory group, demyelinating and axonal group.A twotailed probability value <0.05 was considered significant.

Definition of Important Study Variables Case of GBS
1) Acute onset of bilateral and relatively symmetric flaccid weakness/ paralysis of the limbs with or without involvement of respiratory or cranial nerve-innervated muscles.
2) Decreased or absent deep tendon reflexes at least in affected limbs, monophasic illness pattern, with weakness nadir reached between 12 hours and 28 days, followed by clinical plateau and subsequent improvement, or death.

Recurrence of GBS
A recurrence was defined as two or more episodes that fulfilled the diagnostic criteria for GBS, with a minimum time interval between each episode of 2 months (when fully recovered in between) or 4 months (when only partially recovered).

Asymmetry
Asymmetry in motor deficit demonstrated either clinically or electro physiologically.

Demographic characteristics
During the study period 36 GBS patients were identified.The age of incidence ranged from 19 years to 68 years having a mean age of 35 years.The median age of GBS patients was 34 years (interquartile range 23.5); 61.1% of patients were aged< 40 years.21 (58.3%) of GBS patients were males.The male to female ratio was 1.4: 1 (Figure 1).
Most of the studies were conducted either in children or both adults and children.
In this study, 22 (61.1%)were, < 40 years and 14 (38.8%) were aged > 40 years.The mean age was 35 years.1][12][13] In the present study also, this trend was observed with males constituting 21 (58.3%) of the GBS cases.Rainy (June -September) predominance 14 (38.8%) was noted in our study followed by winter (December-February) 11 (30.5%), even 46 years in demyelinating and 35 years in axonal variant of GBS.Females 7(58.3%) were affected predominantly in demyelinating form and males 13 (72.2%) in axonal form of GBS.CSF protein levels were increased in 27 (81.8%).Similar finding were reported by Bhargava et al. 10 Mean CSF protein was 80.27mg/dl and is comparable to studies by Chio et al. 21and Corston et al. 22 Mortality in GBS is associated with respiratory failure requiring mechanical ventilation that prolongs hospital stay.To detect early respiratory failure, all cases with Hughes grade 4 and those with MRC sum score suggestive of moderate (11-30) and severe (0-10) motor deficit were assessed intensively for early respiratory failure.Battery of tests included were baseline measurement of peripheral saturation (Sao2), single breath count (SBC), peak expiratory flow meter and dysphagia tests.Those with SBC < 15, Sao2 of <95 % and positive dysphagia tests were given oxygen inhalation through nasal cannula, Ryles tube insertion to prevent aspiration pneumonia and early IVIG administration.We modified Hughes grade 5 into two parts, Grade 5A were those who were in respiratory failure, not requiring ventilator or those who can be managed with intensive care (oxygen supplements, ryles tube insertion, management of aspiration pneumonia and early IVIG), other group included those who required mechanical ventilator (cannot be managed conservatively).All our cases 10 (27.7%) who were in Hughes grade 5A were started with IVIG without delay.None of our cases required mechanical ventilation.Plasmapheresis was not considered in most cases due to time constraints in arranging fresh frozen plasma, shifting patients and delay in availability of bed in dialysis unit, requirement of repeated assessment of bleeding parameters and electrolytes, and risk of hypo tension and infection.In present study there was a significant association (P value-0.0035)with low MRC sum score (0-10) at nadir and respiratory failure.Low MRC sum score is an independent risk factor for development of respiratory failure and need for mechanical ventilation.Our findings are in accordance with the study done by Wu X et al. 23 Outcome of patients following IVIG in 32 (88.8%) and plasmapheresis in 1(2.7%) in present study showed complete recovery in 33 (91.6.%) at 6months.This is comparable to previous studies. 10,11,13,20Early arrival (<1 week) to hospital, initiation of intravenous Immunoglobulin and intensive care helped to achieve complete recovery in our study.Axonal form of GBS recovered more slowly than those with demyelinating form but outcome at 12 months appear to be equally favorable in two groups.

CONCLUSION
Males were predominantly affected.Motor weakness was the most common presenting illness.Female preponderance was observed in demyelinating form and male preponderance in axonal form of GBS.AIDP patients were older, while axonal form were younger.Seasonal occurrence predominantly in rainy season was noted.Axonal (AMAN+ AMSAN) form dominated than demyelinating form (AIDP). Peak flow test, peripheral saturation, single breath count, and bulbar weakness may be a predictor of respiratory failure.There was a significant association of Low MRC sum score with respiratory failure.Early diagnosis of respiratory failure and prompt intervention improves patient outcome.Further large sample studies are required to assess respiratory failure and subdivision of Hughes grade 5.

4 ) 5 )
Presence of albuminocytological dissociation (elevation of cerebrospinal fluid (CSF) protein level above laboratory normal value, and CSF total white cell count <50 cells/mm.Absence of an alternative diagnosis for weakness.

Table 3 : Neurological deficit and Guillain-Barre disability score Motor deficits Number (Percentage)
International Journal of Medicine and Public Health, Vol 6, Issue 4, Oct-Dec, 2016