Background and objective: Guillain-Barré syndrome (GBS) is an immune system-mediated polyradiculoneuropathy that accounts for approximately 100,000 new patients per year globally. GBS has also been linked to respiratory failure in 20% to 40% of cases. The objective of this study was to find an association between Erasmus GBS respiratory insufficiency score (EGRIS) and the need for mechanical ventilation. Methods: We conducted our cross-sectional study at department of Neurology, Pakistan Institute of Medical Sciences from November 2019 to February 2020. Sixty patients were selected for this study. Data including demographic profile, variant of GBS, and EGRIS was calculated from all the study participants. Data was entered and analyzed using SPSS version 25.
 Results: All 60 patients enrolled in the study underwent continuous monitoring during admission time. Out of 60 patients, three (5%) endured mechanical ventilation with a mean EGRIS of 4.5. Others who didn\u27t need mechanical ventilation had mean EGRIS of 1.5. The three patients requiring mechanical ventilation were one each from Acute Inflammatory Demyelinating polyradiculoneuropathy, Acute Motor Axonal Neuropathy, and Acute Motor and Sensory Axonal Neuropathy variants of GBS. p-value was non-significant for the presented data.
Conclusion: EGRIS could not meet statistical significance in identifying the requirement of mechanical ventilation for GBS in this study. This may be due to low sample size

Abbasi, Hira

Adil, Malik

Ali, Ijaz

Ali, Sajid

Irfan, Anum

Khalid, Bushra

Khan, Soban

Malik, Waleed

Naik, Samer

Tariq, Muhammad

Waqar, Zaid

English

eCommons@AKU

Pakistan Journal of Neurological Sciences (PJNS) Volume 18 Issue 1 Article 9 3-2023 Exploring The Relationship Between Egris and The Need For Mechanical Ventilation In Guillain Barré Syndrome Bushra Khalid Pakistan Institute of Medical Sciences, Islamabad, Pakistan Zaid Waqar Pakistan Institute of Medical Sciences, Islamabad, Pakistan Soban Khan Pakistan Institute of Medical Sciences, Islamabad Sajid Ali Pakistan Institute of Medical Sciences, Islamabad, Pakistan Ijaz Ali Pakistan Institute of Medical Sciences, Islamabad, Pakistan See next page for additional authors Follow this and additional works at: https://ecommons.aku.edu/pjns  Part of the Neurology Commons Recommended Citation Khalid, Bushra; Waqar, Zaid; Khan, Soban; Ali, Sajid; Ali, Ijaz; Tariq, Muhammad; Irfan, Anum; Malik, Waleed; Naik, Samer; Adil, Malik; and Abbasi, Hira (2023) "Exploring The Relationship Between Egris and The Need For Mechanical Ventilation In Guillain Barré Syndrome," Pakistan Journal of Neurological Sciences (PJNS): Vol. 18: Iss. 1, Article 9. Available at: https://ecommons.aku.edu/pjns/vol18/iss1/9 Exploring The Relationship Between Egris and The Need For Mechanical Ventilation In Guillain Barré Syndrome Authors Bushra Khalid, Zaid Waqar, Soban Khan, Sajid Ali, Ijaz Ali, Muhammad Tariq, Anum Irfan, Waleed Malik, Samer Naik, Malik Adil, and Hira Abbasi This original article is available in Pakistan Journal of Neurological Sciences (PJNS): https://ecommons.aku.edu/pjns/vol18/iss1/9 2 6P A K I S T A N  J O U R N A L  O F  N E U R O L O G I C A L  S C I E N C E S V O L .  1 8  ( 1 )  J A N - M A R  2 0 2 3 https://doi.org/10.56310/pjns.v18i01.253INTRODUCTION Guillain-Barré syndrome (GBS) is an immune system-mediated polyradiculoneuropathy that accounts for approximately 100,000 new patients per year globally.1 GBS is also linked to respiratory failure in 20% to 40% of cases.2 To predict respiratory muscle involvement is vital to avoid respiratory distress and prioritize cases in the intensive care unit (ICU). Optimum management is required in GBS to avoid respiratory failure so prediction of its clinical features and in-time intubation of a patient is required. Swift intervention is linked with decreased rates of complications and improved results for the patient.3 In most cases, acute onset of neurological symptoms follows an infective disease subsequently causing advanced limb weakness that could progress for four months before hitting a plateau. Various infections are linked to GBS. Among these Campylobacter jejuni is found to be the most common and is reported by various researchers. For C jejuni associated GBS, there are strong pieces of evidence that suggest that molecular mimicry is present among nerve endings and EXPLORING THE RELATIONSHIP BETWEEN EGRIS AND THE NEED FOR MECHANICAL VENTILATION IN GUILLAIN BARRÉ SYNDROMEBushra Khalid1, Zaid Waqar1, Soban Khan1, Sajid Ali1, Ijaz Ali1, Muhammad Tariq1, Anum Irfan1, Waleed Malik1, Samer Naik1, Malik Adil1, Hira Abbasi11.Pakistan Institute of Medical Sciences, IslamabadABSTRACTBackground and objective:Guillain-Barré syndrome (GBS) is an immune system-mediated polyradiculoneuropathy that accounts for approximately 100,000 new patients per year globally. GBS has also been linked to respiratory failure in 20% to 40% of cases. The objective of this study was to find an association between Erasmus GBS respiratory insufficiency score (EGRIS) and the need for mechanical ventilation.Methods:We conducted our cross-sectional study at department of Neurology, Pakistan Institute of Medical Sciences from November 2019 to February 2020. Sixty patients were selected for this study. Data including demographic profile, variant of GBS, and EGRIS was calculated from all the study participants. Data was entered and analyzed using SPSS version 25.Results:All 60 patients enrolled in the study underwent continuous monitoring during admission time. Out of 60 patients, three (5%) endured mechanical ventilation with a mean EGRIS of 4.5. Others who didn't need mechanical ventilation had mean EGRIS of 1.5. The three patients requiring mechanical ventilation were one each from Acute Inflammatory Demyelinating polyradiculoneuropathy, Acute Motor Axonal Neuropathy, and Acute Motor and Sensory Axonal Neuropathy variants of GBS. p-value was non-significant for the presented data.Conclusion:EGRIS could not meet statistical significance in identifying the requirement of mechanical ventilation for GBS in this study. This may be due to low sample size.Keywords: EGRIS, Mechanical ventilation, GBS, NeuropathyO R I G I N A L  A R T I C L ECorresponding author: Zaid Waqar Pakistan Institute of Medical Sciences, Islamabad Email: chikky789@gmail.comDate of submission: January 1, 2023 Date of revision: March 25, 2023 Date of acceptance: March 25, 20232 7P A K I S T A N  J O U R N A L  O F  N E U R O L O G I C A L  S C I E N C E S V O L .  1 8  ( 1 )  J A N - M A R  2 0 2 3microbial antigens, causing the development of GBS.5The classic manifestation of GBS does not generally cause a diagnostic challenge, but atypical forms are overlooked when not examined for. For assistance in the diagnosis, nerve conduction studies are carried out to detect polyradiculoneuropathy and cerebrospinal fluid examination could reveal albumin cytological dissociation. Yet diagnosis should never solely depend on these tests because both of these could be normal in the initial stage of the syndrome.6 Patients suffering from GBS need rigorous monitoring for disease advancement, specifically for autonomic dysfunction, respiratory insufficiency, and bulbar weakness. Predictive scales for the prediction of patient outcomes and to classify treatment have been developed. Plasma exchange and intravenous immunoglobulin are the only two approved immunotherapies that can speed up recovery in GBS. Even after treatment via standard immune therapies, approximately 5% of patients die, while approximately 20% of patients cannot not walk unassisted at least for 12 months after the disease progression.6In studies done by various Intergovernmental Organizations (IGOs), an antecedent incident was identified in 76% of cases, primarily nose, sinuses, mouth, pharynx, and larynx infections (35%) in East Asia and Southeast Asia, North America, and Europe, while intestinal infection (27%) was more prevalent in Bangladesh.7 GBS has also been linked to certain vaccination sand with immune checkpoint inhibitor therapy.8,9 Moreover less frequent triggers consist of ganglioside application and surgery. In 1991, McKhann and colleagues researched the seasonal epidemic of acute equivocal Chinese paralysis syndrome.10 This hallmark research afterward led to the identification of acute motor axonal neuropathy (AMAN) and acute motor-sensory axonal neuropathy (AMSAN) as clinical variants of GBS caused by C jejuni infection. Up till now, different types of GBS variants had been identified. Most prevalent of these are AMSAN and AMAN (axonal types) and acute inflammatory demyelinating polyneuropathy (AIDP). Erasmus GBS Respiratory Insufficiency Score (EGRIS) gave 91% predictive value for mechanical ventilation (MV) at the time of admission for seven patients in one study.11 Present study was conducted to find an association of EGRIS with the requirement of mechanical ventilation (MV) and to verify its effectiveness as a predictive model.METHODSStudy design: Cross-sectional study.Place and duration of study: This study was conducted for four months (from November 2019 to February 2020) in the Department of Neurology, Pakistan Institute of Medical sciences.Sample size: Sixty, calculated using WHO sample size calculator.Sampling technique: Non-probability consecutive sampling.Inclusion Criteria: Patients aged above 16 years with acute onset ascending paralysis with NCS evidence of acute neuropathy and/or CSF analysis showing albuminocytologic dissociation.Exclusion criteria:  • Patients with other systemic diseases that may   cause neuropathy such as uremia, malignancy. • Patients with pyramidal signs such as up-going    plantar reflexes. • Patients with clinical/radiological evidence of    myelopathyData collection: After taking informed consent. all the 60 patients’ data was collected including demographics, clinical diagnosis and GBS variant. and need for mechanical ventilation. EGRIS was calculated on all patients upon admission to Department of Neurology. The EGRIS measures the chances of MV in GBS during the first week of monitoring. It is calculated on 3 major criteria. Each criterion is considered and allotted a separate score; the addition of these scores provides total EGRIS for a patient (between 0 and 7). A score of 0–2 Predicts a low chance of mechanical ventilation (MV), 3–4 shows a moderate chance for MV and ≥5 depicts an elevated chance for MV.Data analysis: The data was analyzed through SPSS 22.0.Ethical considerations: The research protocol was approved by the PIMS Ethics Review committee.2 8P A K I S T A N  J O U R N A L  O F  N E U R O L O G I C A L  S C I E N C E S V O L .  1 8  ( 1 )  J A N - M A R  2 0 2 3RESULTSAll patients were admitted to neurology ward and underwent monitoring for clinical deterioration and respiratory distress. One predictive factor of a patient’s need for early MV was the number of days between the onset of motor symptoms and hospitalization. Out of 60 patients, three (5%) went on mechanical ventilation with a mean EGRIS of 4.5. While others who didn't need mechanical ventilation had EGRIS mean of 1.5. The three patients requiring MV were one from each from AIDP, AMAN, and AMSAN. In our study 31 patients were male and 29 were female Figure 1 is a pie chart showing the age distribution in our patients with the highest number found in age group 36-45 years.The highest number of patients were suffering from AMAN. The number of patients suffering from AMAN was 33 (54.1%), the number of patients suffering from ADP was 15 (24.6%) and the number of patients suffering from AMSAN was 12 (19.7%).  The number of patients suffering from seven days or more was 36 (59.0%), the number of patients suffering from four to seven days was 20 (32.8%) and the number of patients suffering from less than three days was 4 (6.6%). When EGRIS was calculated for patients included in the study, 75.4% of cases had a score ≥3. Each of the three variants had the same 1.66% for the need of MV with a cumulative percentage of 5%. Details of the frequency of MV requirements in different GBS variants are given in Table 1.Figure 1: Illustration of age-wise frequency of patients2 9P A K I S T A N  J O U R N A L  O F  N E U R O L O G I C A L  S C I E N C E S V O L .  1 8  ( 1 )  J A N - M A R  2 0 2 3AIDP: Acute inflammatory demyelinating polyradiculoneuropathyAMAN: Acute motor axonal neuropathyAMSAN: Acute motor and sensory axonal neuropathyStatistical analysis of data didn’t show any significant relationship between EGRIS  and the need for MV (Table 2)DISCUSSIONGBS is a severe, immune system-mediated attack on the peripheral nervous system which results in progressive motor weakness and/or sensory loss. Its incidence rate is 0.4–4.0 per 100,000 people/year and after the decrease in the rate of polio cases now it is the largest reason for acute neuromuscular paralysis.12 On the basis of different characteristics like clinical, pathological, and electrophysiological it is declared as a heterogeneous disorder that includes different subtypes: AIDP, AMAN, AMSAN, and Miller-Fischer syndrome.13 EGRIS is a predictive model to estimate the early requirement of MV.14 GBS has many electrophysiological subtypes and predominant phenotypes in various regions so the disease severity and the EGRIS  also changes with change in the region.15The majority of patients included in this study were male belonging to an age group of 36 to 45 years. Globally, researches from Latin American, and European literature reports its prevalence majorly in 3 0P A K I S T A N  J O U R N A L  O F  N E U R O L O G I C A L  S C I E N C E S V O L .  1 8  ( 1 )  J A N - M A R  2 0 2 3Limitations: This study showed higher EGRIS for patients needing mechanical ventilation but due to smaller sample size it didn’t reach any statistical significance.CONCLUSIONEGRIS could not significantly predict the need for MV in GBS patients of this study. This was likely due to the small sample size. More studies are needed with larger samples to correctly analyze the utility of EGRIS.male and aged patients.15 In populations like Asian, and Latin American lineage AMAN was reported as a prevalent variant.15,16 In the present study AMAN was more prevalent than AMSAN and AIDP which makes it also a prevalent variant in the Pakistani population. Of the cases in the present study, only 5% of patients required MV, which is a much lower percentage as compared to 20–25% reported globally.16Two major causes of mortality in GBS are respiratory failure and autonomic dysfunction.17,18 Treatment for GBS aims to reduce mortality and hasten the recovery from acute neuropathy that lead to paralysis. The two main treatment options apart from supportive care are therapeutic plasma-pharesis and intravenous immune globulins.19 These treatments aim to reduce the respiratory muscle involvement and improve the survival. There are various clinical and neuro-physiological factors whose presence warns of the development of respiratory weakness.20 For this purpose various prediction scores have been established, one of them is EGRIS. It aims to predict the development of respiratory insufficiency in GBS patients.In the present study, it was noticed that 74.5% of patients' EGRIS was less than or equal to 3. Hence, they did not require MV. Only 3 patients whose EGRIS was greater than or equal to 5 required MV. Our results correlate with another research where higher EGRIS was associated with the early requirement of MV. Researchers from Japan and Malaysia reported the same evidence by depicting statistically significant differences in EGRIS in patients who required and did not require MV. Correspondingly, a team of researchers in South Korea reported a positive correlation between the chances of MV and EGRIS.20REFERENCES1. Sejvar JJ, Baughman AL, Wise M, Morgan OW.   Population incidence of Guillain-Barré syndrome:   a systematic review and meta-analysis. Neuroepi  demiology. 2011;36(2):123-133.     doi:10.1159/0003247102. Lawn ND, Fletcher DD, Henderson RD, Wolter  TD, Wijdicks EF. Anticipating mechanical ventila  tion in Guillain-Barré syndrome. Arch Neurol.    2001;58(6):893-898. doi:10.1001/arch   neur.58.6.8933. Wijdicks EF, Borel CO. Respiratory management  in acute neurologic illness. 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Funding disclosure: NilAuthors’ contribution:Bushra Khalid; Concept, data analysis, manuscript writing, manuscript revisionZaid Waqar; Concept, data collection, data analysis, manuscript writing, Soban Khan; Data collection, manuscript writing, manuscript revision Sajid Ali; Data collection, data analysis, manuscript writing Ijaz Ali; Data collection, data analysis, manuscript writingMuhammad Tariq; Data collection, data analysis, manuscript writingAnum Irfan; Data collection, data analysis, manuscript writingWaleed Malik; Data collection, data analysis, manuscript writingSamer Naik; Data collection, data analysis, manuscript writingMalik Adil; Concept and design, manuscript revisionHira Abbasi; Concept and design, manuscript revisionAll the authors have approved the final version of the article, and agree to be accountable for all aspects of the work.

Exploring The Relationship Between Egris and The Need For Mechanical Ventilation In Guillain Barré Syndrome

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Exploring The Relationship Between Egris and The Need For Mechanical Ventilation In Guillain Barré Syndrome

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