|Year : 2022 | Volume
| Issue : 3 | Page : 163-169
Guillain–Barré Syndrome: Clinical Profile and Electrodiagnostic Subtype Spectrum from a Tertiary Care Hospital in Eastern India
Sanjeev Kumar1, Priyanka Rani2, Janardan Sharma1, Anand Kumar Rai1
1 Department of Neurology, Indira Gandhi Institute of Medical Sciences, Patna, Bihar, India
2 Department of Obstetrics and Gynecology, Netaji Subhas Medical College and Hospital, Patna, Bihar, India
|Date of Submission||19-Apr-2022|
|Date of Decision||22-May-2022|
|Date of Acceptance||26-May-2022|
|Date of Web Publication||3-Oct-2022|
MD, DM Sanjeev Kumar
Assistant Professor, Department of Neurology, Indira Gandhi Institute of Medical Sciences, Patna, Bihar
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Background: Guillain–Barré syndrome (GBS) is an immune-mediated acute polyradiculoneuropathy with different subtypes, clinical features, and demographics. Nerve conduction study is important in differentiating axonal and demyelinating form of GBS. Diagnosis of various subtypes is essential as they have different pathophysiology and prognosis. Objective: The study was aimed to evaluate the different subtype spectrum of GBS in our patient population cohort and to look for the clinical features, demographics, and electrophysiological profile variations among the patients with GBS. Materials and Methods: We evaluated clinical spectrum and electrodiagnostic parameters of the admitted patients in Department of Neurology of our tertiary care center between September 2019 and April 2022 with clinical diagnosis of GBS. It was a quantitative descriptive cross-sectional study. Results: Out of 49 study participants, 63.82% patients had axonal form while 36.2% of patients had acute inflammatory demyelination polyneuropathy by applying Hadden criteria. Statistically lower single breath count (SBC) (median 10.5; P < 0.0001) at admission was observed in ventilated patients compared to nonventilated patients of GBS. Conclusion: Axonal form is the most common subtype of GBS in our study cohort. SBC at admission could be an important bedside tool to predict requirement of ventilatory support in admitted patients of GBS.
Keywords: AIDP, AMAN, electrodiagnostic study, GBS, single breath count
|How to cite this article:|
Kumar S, Rani P, Sharma J, Rai AK. Guillain–Barré Syndrome: Clinical Profile and Electrodiagnostic Subtype Spectrum from a Tertiary Care Hospital in Eastern India. Int J Nutr Pharmacol Neurol Dis 2022;12:163-9
|How to cite this URL:|
Kumar S, Rani P, Sharma J, Rai AK. Guillain–Barré Syndrome: Clinical Profile and Electrodiagnostic Subtype Spectrum from a Tertiary Care Hospital in Eastern India. Int J Nutr Pharmacol Neurol Dis [serial online] 2022 [cited 2022 Dec 5];12:163-9. Available from: https://www.ijnpnd.com/text.asp?2022/12/3/163/357213
| Introduction|| |
Guillain–Barré syndrome (GBS) presents with an acute flaccid weakness of both upper and lower limbs with or without sensory or autonomic disturbances. Clinically, patients had areflexia or hyporeflexia without cerebrospinal fluid (CSF) pleocytosis. Worldwide incidence of GBS is 1.2 to 3/100,000 population per year. Annual incidence of GBS increased with age from 0.8 in those <18 years old to 3.2 in those >60 years old. Up to 40% of patients of GBS had respiratory muscle involvement., Based on previous studies, factors predicting the need of endotracheal intubation are: shorter disease duration for nadir (<7 days), diminished gag reflex, inability to cough properly, inability to stand, and inability to lift the head or elbows. These factors can anticipate the need of mechanical ventilation in patients with GBS.
Nerve conduction studies (NCS) are an important tool in confirming the diagnosis and distinguishing various subtypes of GBS: acute motor axonal neuropathy (AMAN), acute inflammatory demyelinating polyradiculoneuropathy (AIDP), and acute motor sensory axonal neuropathy (AMSAN). As in Western population, AIDP is more prevalent; most electrophysiological diagnostic criteria had emphasized the diagnosis of AIDP.,
Our institute is a tertiary care referral center in the eastern India with referrals from different parts of country. The study was aimed to evaluate the different subtype spectrum of GBS in our patient population cohort and to look for the clinical features, demographics, and electrophysiological profile variations among the patients with GBS.
| Materials and Methods|| |
This study was designed as a prospective descriptive cross-sectional study. Patients admitted in Department of Neurology with weakness of all four limbs with disease progression <4 weeks duration were included in this study. A thorough clinical examination was performed and secondary causes of weakness were excluded like periodic paralysis, porphyria, viral myositis, and toxic neuropathy.
Analysis of nerve conduction studies
As per our standard electrophysiological laboratory protocol, all study patients who were admitted in Department of Neurology between September 2019 and April 2022 underwent nerve conduction studies with recording of at least eight motor and six sensory nerves conduction using standard NCS techniques. Nerve conduction study was done within 4 weeks of disease onset. Various parameters were determined including distal motor and sensory latencies, compound muscle action potentials, motor and sensory conduction velocities, latency of F wave, H reflex, and sensory nerve action potentials (SNAPs). Temperature of skin was kept at >32°C. Sensory abnormality was detected as per our laboratory standard normative cutoff value. Each study patient’s data were entered into an appropriate computer program and the Hadden et al.’s criteria were applied to diagnose GBS and its different subtypes as shown in [Figure 1].
|Figure 1 Criteria used for electrodiagnostic diagnosis of GBS. Source: Adapted from Hadden et al. with permission from John Wiley and Sons.|
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Baseline clinical and laboratory characteristics regarding clinical features, Hughes disability scoring, CSF analysis, and preceding infections were obtained during hospital stay.
| Results|| |
We prospectively included 49 patients in our study − 40 adults and nine children; 38 males and 11 females were there. About 47 patients underwent nerve conduction study as we did not have facility to perform NCS in intensive care unit setting. Baseline characteristics are shown in [Table 1].
Hughes disability functional grading scale was applied at admission and at discharge. This distribution pattern is demonstrated in [Figure 2].
|Figure 2 Hughes functional grade of GBS patients at admission and discharge.|
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Ventilatory support and single breath count at admission
About 16.32% (n = 8/49) patients required ventilatory support in our hospital cohort. And two patients had fatal outcome.
The median single breath count (SBC) at admission in nonventilated patients was 24 while median SBC of patients on ventilatory support was 10.5. Admission mean SBC of patients without ventilatory support was statistically high compared to patients who required ventilatory support (P < 0.0001). It is illustrated in [Table 2].
|Table 2 Showing mean admission SBC in ventilated and nonventilated GBS patients|
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About 87.75% (n = 43/49) of patients underwent CSF study in which, 41 patients had cytoalbuminologic dissociation. The mean CSF protein was 138.86 mg/dL. One patient with CSF protein of 389.6 mg/dL had bilateral papilledema on clinical examination.
Cranial nerve abnormality
Bilateral lower seventh cranial nerve palsy was seen in 40.81% (n = 20/49) of patients. Bulbar weakness was seen in 14.28% (n = 7/49) of patients. Papilledema was seen in one patient.
Seasonal variation trends
Our study showed 55.1% of cases in winter season (November to February), followed by rainy season, 34.69% (July to October). Least number of cases were seen in summer season, 10.20%. This is shown in [Figure 3].
As we have limitations of providing plasma exchange, the other various modalities of treatment received included: intravenous immunoglobulin (IVIG) in 71.42% (n = 35), and no specific treatment in 28.57% (n = 14) of patients, which is demonstrated in [Figure 4] as a pie chart.
|Figure 4 Pie chart showing various treatment modalities received during hospital stay in GBS patients.|
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The median duration between the onsets of illness to baseline electrodiagnostic studies was 8 days.
As there was no facility in ICU for NCS test, we performed nerve conduction studies only in 47 patients.
Total of 470 nerves were evaluated: 94 each median, ulnar, tibial, perineal, and sural nerves. Conduction of motor nerves was measured between two segments in the median nerves, ulnar nerves, tibial nerves, and the peroneal nerves.
Hadden et al. criteria were applied to subdivide various GBS types. As per these criteria, 28 (59.57%) patients had AMAN variant, 17 (36.17%) patients had AIDP variant, and two (4.25%) patients had AMSAN subtype. Motor conduction block in at least two nerves was seen in five (10.63%) patients. H-reflex abnormality was seen in 89.36% (42/47) of patients. Distribution of various GBS types is shown in [Table 3].
Children versus adults
Out of the nine children, four (44.43%) had AMAN as compared to adults, where 24 (63.15%; 24/38) had AMAN subtype (P = 0.23). Five children had AIDP (55.55%), whereas 12 (31.57%) adults had AIDP (P = 0.18). Two (5.2%) adults had AMSAN, whereas no children had AMSAN. Distribution of various subtypes of GBS among children and adults is shown in [Table 4].
|Table 4 Showing various subtypes of GBS among adults and pediatric patients|
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Sensory nerve conduction abnormalities
Clinical examination revealed sensory changes in 10.20% of patients, while electrodiagnostic studies demonstrated abnormalities in sensory nerve conduction in 40.42% (19/47) of patients. Normal sural nerve SNAPs with abnormal median nerve SNAPs were seen in 27.65% (13/47) of patients. Sural sparing pattern among AIDP was seen in 76.47% of patients. It was only found in AIDP patients. Characteristic sensory abnormalities are shown in [Table 5].
|Table 5 Showing sensory abnormality and sural sparing pattern among GBS subtypes|
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| Discussion|| |
In this study, we prospectively investigated various clinical features and performed nerve conduction studies at admission. GBS disability score (Hughes score) was performed at the time of admission and at the time of discharge.
In this study, mean age of our cohort was 32 years. A study done by Sudulagunta et al. showed mean age of 43 years in their study. Yoshikawa showed an average age of presentation at 39 years with males 1.5 times more affected than the females. In our study as well, males (77.55%) were more affected than females (22.44%).
About 36.73% of GBS patients gave history of antecedent events. Serological studies reveal evidence of antecedent infection in about 30% to 50% of cases.,,,
We graded clinical severity at admission according to Hughes et al., severity scale, ranging from zero to six, and at the time of discharge. Higher scoring showed high disease severity and poorer outcome. Our study showed Hughes grade 4 in 44.89% of patients at admission followed by Hughes grade 3 in 28.57%. Maximum number of patients discharged at Hughes grade 3 (n = 17; 34.69%).
Our study showed high proportion of cases in winter season with maximum in October month. A study by Shrivastava et al. showed higher percentage of cases during summer season with maximum in July. A study by Webb et al. showed higher number of cases during winter season due to greater number of upper respiratory infection in Western population during winter season. Our study had variation partly because of COVID pandemic.
Cranial nerve abnormality showed bilateral lower seventh nerve cranial palsy in 41% of patients, while bulbar weakness was seen in 14%. A study by Gurwood and Drake showed unilateral or bilateral lower seventh nerve cranial palsy in 50% of cases followed by bulbar muscle weakness.
CSF study showed cytoalbuminologic dissociation in 83.7% of patients. The mean CSF protein was 138.86 mg/dL. A study by Rath et al. showed elevated CSF protein in 72% of cases.
Our cohort showed ventilatory requirement in 16% of patients. A study by Lawn et al. showed that up to 30% of GBS patients required ventilatory support. SBC at admission was significantly lower in such cohort compared to nonventilated patients. A study by Kalita et al. showed that GBS patients with SBC of 5 in need of mechanical ventilation could be predicted with 90.6% of sensitivity and specificity of 95.2%.
GBS subclassification into various subtypes is of importance as its pathogenesis, severity, and prognosis may be different between the various subtypes., Geographical differences had shown that the incidence of AIDP is more frequent in the Western population while axonal variant is more common in the Asian population., Diagnosis of AMAN and AMSAN variants requires the accurate identification of AIDP. A study conducted by a Dutch group presented AIDP as the most widely recognized subtype, and most electrophysiological criteria depended on the accurate identification of peripheral nerve demyelination. However, there is disagreement among different researchers for the detection of acute demyelination in peripheral nerves. The two most frequently used electrophysiological criteria, Hadden et al.’s and Rajabally et al.’s, were used to classify different subtypes of GBS. The clinical severity rather than time from symptom onset to electrophysiological evaluation correlated with fulfillment of criteria for a demyelination or axonal subtype.
In our study cohort, 28 (59.57%) patients had AMAN variant, 17 (36.17%) patients had AIDP variant, and two (4.25%) patients had AMSAN subtype. Motor conduction block in at least two nerves was seen in five (10.63%) patients. H-reflex abnormality was seen in 89.36% (42/47) of patients. According a study conducted by Alexander et al., 44.3% patients had axonal subtype of the disease, 30.4% had AMAN, and 13.6% had AMSAN, 38.2% patients had AIDP, and 20 (17.4%) patients remained equivocal.
In axonal type of GBS, there is activation of complement system and antibody-mediated nodal and paranodal involvement resulting in conduction failure. This can manifest as a reversible conduction failure, conduction slowing, and isolated F-wave absence. Classically, this results in axonal degeneration.
An early accurate electrophysiological diagnosis becomes important tool in the management of GBS. IVIG could therapeutically be more effective in settings of complement-mediated damage. Some preliminary studies had suggested that IVIG is better than plasmapheresis in AMAN variant of GBS.
Among AIDP, sural nerve sparing pattern was seen in 76.47% of patients. Classically, a sural sensory nerve sparing pattern is demonstrated only in AIDP and is a specific electrophysiological feature that can discriminate AIDP from axonal form of GBS. In a study of 73 patients of GBS, Derksen et al. observed that sural nerve sparing pattern was most unique and specific electrodiagnostic feature suggestive of AIDP. Our study also found similar sural nerve sparing pattern only seen in AIDP, and it is very specific for diagnosis of AIDP.
AMAN subtype accounted for 44.43% of pediatric GBS, whereas 66.63% of the adults had AMAN subtype. In adults, AIDP accounted for 31.75 % of the cases, whereas 55.56 % of the children had AIDP.
Alexander et al. showed similar trend in AMAN variant, whereas opposite trends were seen in AIDP subtype. This difference could be due to smaller sample size in pediatric population.
Indian studies have shown AIDP to be more common. In a study from northern India (SGPGI, Lucknow), the distribution of GBS subtypes was: AIDP in 86.3%, AMAN in 7.8%, and AMSAN in 6.7% patients. In a south Indian study, GBS distribution pattern showed AIDP in 85.2% patients, axonal variants in 10.6% patients, while 4.2% patients remained unclassified. Alexander et al., from south Indian population, showed similar trends as of our study. The difference between our study and the other two Indian studies is most probably due to the different population cohort, demographics, and smaller sample size.
The limitations of our study are: cross-sectional electrophysiological study, absence of serology for antecedent infections, and smaller sample size, probably due to COVID pandemic.
| Conclusions and Future Scope|| |
Axonal form is the most common type of GBS in our study cohort. Serial nerve conduction study is warranted for further subtype classification. SBC at admission could be an important bedside tool to predict requirement of ventilatory support in admitted patients of GBS. Antiganglioside antibody can be assessed in further studies to differentiate axonal variants from AIDP. Different electrophysiological criteria can be applied to determine the sensitivity of each criterion.
Authors would like to thank Dr Priyanka Rani for her help in preparation of this manuscript and various statistical analyses. We would also like to thank our patients and working colleague for their continuous support for this study.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]
[Table 1], [Table 2], [Table 3], [Table 4], [Table 5]