Regional variation of Guillain-Barré syndrome

Alex Y. Doets, Christine Verboon, Bianca Van Den Berg, Thomas Harbo, David R. Cornblath, Hugh J. Willison, Zhahirul Islam, Shahram Attarian, Fabio A. Barroso, Kathleen Bateman, Luana Benedetti, Peter Van Den Bergh, Carlos Casasnovas, Guido Cavaletti, Govindsinh Chavada, Kristl G. Claeys, Efthimios Dardiotis, Amy Davidson, Pieter A. Van Doorn, Tom E. Feasby & 24 others Giuliana Galassi, Kenneth C. Gorson, Hans Peter Hartung, Sung Tsang Hsieh, Richard A.C. Hughes, Isabel Illa, Badrul Islam, Susumu Kusunoki, Satoshi Kuwabara, Helmar C. Lehmann, James A.L. Miller, Quazi Deen Mohammad, Soledad Monges, Eduardo Nobile Orazio, Julio Pardo, Yann Pereon, Simon Rinaldi, Luis Querol, Stephen W. Reddel, Ricardo C. Reisin, Nortina Shahrizaila, Soren H. Sindrup, Waheed Waqar, Bart C. Jacobs

Research output: Contribution to journalArticle

6 Citations (Scopus)

Abstract

Guillain-Barré syndrome is a heterogeneous disorder regarding the clinical presentation, electrophysiological subtype and outcome. Previous single country reports indicate that Guillain-Barré syndrome may differ among regions, but no systematic comparative studies have been conducted. Comparative studies are required to identify factors determining disease susceptibility, variation and prognosis, and to improve diagnostic criteria. The International Guillain-Barré Syndrome Outcome Study is a prospective, observational cohort study including all patients within the diagnostic spectrum, aiming to describe the heterogeneity of Guillain-Barré syndrome worldwide. The current study was based on the first 1000 inclusions with a follow-up of at least 1 year and confirmed the variation in clinical presentation, course and outcome between patients. The full clinical spectrum of Guillain-Barré syndrome was observed in patients from all countries participating in the International Guillain-Barré Syndrome Outcome Study, but the frequency of variants differed between regions. We compared three regions based on geography, income and previous reports of Guillain-Barré syndrome subtypes: Europe/Americas', Asia' (without Bangladesh), and Bangladesh'. We excluded 75 (8%) patients because of alternative diagnoses, protocol violations, or missing data. The predominant clinical variant was sensorimotor in Europe/Americas (n = 387/562, 69%) and Asia (n = 27/63, 43%), and pure motor in Bangladesh (n = 74/107, 69%). Miller Fisher syndrome and Miller Fisher-Guillain-Barré overlap syndrome were more common in Asia (n = 14/63, 22%) than in the other two regions (Europe/Americas: n = 64/562, 11%; Bangladesh: n = 1/107, 1%) (P < 0.001). The predominant electrophysiological subtype was demyelinating in all regions (Europe/Americas: n = 312/573, 55%; Asia: n = 29/65, 45%; Bangladesh: n = 38/94, 40%). The axonal subtype occurred more often in Bangladesh (n = 34/94, 36%) than in Europe/Americas (n = 33/573, 6%) and other Asian countries (n = 4/65, 6%) (P < 0.001). In all regions, patients with the axonal subtype were younger, had fewer sensory deficits, and showed a trend towards poorer recovery compared to patients with the demyelinating subtype. The proportion of patients able to walk unaided after 1 year varied between Asia (n = 31/34, 91%), Europe/Americas (n = 334/404, 83%) and Bangladesh (n = 67/97, 69%) (P = 0.003). A similar variation was seen for mortality, being higher in Bangladesh (n = 19/114, 17%) than in Europe/Americas (n = 23/486, 5%) and Asia (n = 1/45, 2%) (P < 0.001). This study showed that factors related to geography have a major influence on clinical phenotype, disease severity, electrophysiological subtype, and outcome of Guillain-Barré syndrome.

Original languageEnglish
Pages (from-to)2866-2877
Number of pages12
JournalBrain
Volume141
Issue number10
DOIs
Publication statusPublished - Oct 1 2018

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Bangladesh
Geography
Miller Fisher Syndrome
Outcome Assessment (Health Care)
Disease Susceptibility
Observational Studies
Cohort Studies
Phenotype
Mortality

Keywords

  • axonal degeneration
  • clinical course
  • demyelination
  • outcome
  • polyradiculoneuropathy

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Doets, A. Y., Verboon, C., Van Den Berg, B., Harbo, T., Cornblath, D. R., Willison, H. J., ... Jacobs, B. C. (2018). Regional variation of Guillain-Barré syndrome. Brain, 141(10), 2866-2877. https://doi.org/10.1093/brain/awy232

Regional variation of Guillain-Barré syndrome. / Doets, Alex Y.; Verboon, Christine; Van Den Berg, Bianca; Harbo, Thomas; Cornblath, David R.; Willison, Hugh J.; Islam, Zhahirul; Attarian, Shahram; Barroso, Fabio A.; Bateman, Kathleen; Benedetti, Luana; Van Den Bergh, Peter; Casasnovas, Carlos; Cavaletti, Guido; Chavada, Govindsinh; Claeys, Kristl G.; Dardiotis, Efthimios; Davidson, Amy; Van Doorn, Pieter A.; Feasby, Tom E.; Galassi, Giuliana; Gorson, Kenneth C.; Hartung, Hans Peter; Hsieh, Sung Tsang; Hughes, Richard A.C.; Illa, Isabel; Islam, Badrul; Kusunoki, Susumu; Kuwabara, Satoshi; Lehmann, Helmar C.; Miller, James A.L.; Mohammad, Quazi Deen; Monges, Soledad; Nobile Orazio, Eduardo; Pardo, Julio; Pereon, Yann; Rinaldi, Simon; Querol, Luis; Reddel, Stephen W.; Reisin, Ricardo C.; Shahrizaila, Nortina; Sindrup, Soren H.; Waqar, Waheed; Jacobs, Bart C.

In: Brain, Vol. 141, No. 10, 01.10.2018, p. 2866-2877.

Research output: Contribution to journalArticle

Doets, AY, Verboon, C, Van Den Berg, B, Harbo, T, Cornblath, DR, Willison, HJ, Islam, Z, Attarian, S, Barroso, FA, Bateman, K, Benedetti, L, Van Den Bergh, P, Casasnovas, C, Cavaletti, G, Chavada, G, Claeys, KG, Dardiotis, E, Davidson, A, Van Doorn, PA, Feasby, TE, Galassi, G, Gorson, KC, Hartung, HP, Hsieh, ST, Hughes, RAC, Illa, I, Islam, B, Kusunoki, S, Kuwabara, S, Lehmann, HC, Miller, JAL, Mohammad, QD, Monges, S, Nobile Orazio, E, Pardo, J, Pereon, Y, Rinaldi, S, Querol, L, Reddel, SW, Reisin, RC, Shahrizaila, N, Sindrup, SH, Waqar, W & Jacobs, BC 2018, 'Regional variation of Guillain-Barré syndrome', Brain, vol. 141, no. 10, pp. 2866-2877. https://doi.org/10.1093/brain/awy232
Doets AY, Verboon C, Van Den Berg B, Harbo T, Cornblath DR, Willison HJ et al. Regional variation of Guillain-Barré syndrome. Brain. 2018 Oct 1;141(10):2866-2877. https://doi.org/10.1093/brain/awy232
Doets, Alex Y. ; Verboon, Christine ; Van Den Berg, Bianca ; Harbo, Thomas ; Cornblath, David R. ; Willison, Hugh J. ; Islam, Zhahirul ; Attarian, Shahram ; Barroso, Fabio A. ; Bateman, Kathleen ; Benedetti, Luana ; Van Den Bergh, Peter ; Casasnovas, Carlos ; Cavaletti, Guido ; Chavada, Govindsinh ; Claeys, Kristl G. ; Dardiotis, Efthimios ; Davidson, Amy ; Van Doorn, Pieter A. ; Feasby, Tom E. ; Galassi, Giuliana ; Gorson, Kenneth C. ; Hartung, Hans Peter ; Hsieh, Sung Tsang ; Hughes, Richard A.C. ; Illa, Isabel ; Islam, Badrul ; Kusunoki, Susumu ; Kuwabara, Satoshi ; Lehmann, Helmar C. ; Miller, James A.L. ; Mohammad, Quazi Deen ; Monges, Soledad ; Nobile Orazio, Eduardo ; Pardo, Julio ; Pereon, Yann ; Rinaldi, Simon ; Querol, Luis ; Reddel, Stephen W. ; Reisin, Ricardo C. ; Shahrizaila, Nortina ; Sindrup, Soren H. ; Waqar, Waheed ; Jacobs, Bart C. / Regional variation of Guillain-Barré syndrome. In: Brain. 2018 ; Vol. 141, No. 10. pp. 2866-2877.
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title = "Regional variation of Guillain-Barr{\'e} syndrome",
abstract = "Guillain-Barr{\'e} syndrome is a heterogeneous disorder regarding the clinical presentation, electrophysiological subtype and outcome. Previous single country reports indicate that Guillain-Barr{\'e} syndrome may differ among regions, but no systematic comparative studies have been conducted. Comparative studies are required to identify factors determining disease susceptibility, variation and prognosis, and to improve diagnostic criteria. The International Guillain-Barr{\'e} Syndrome Outcome Study is a prospective, observational cohort study including all patients within the diagnostic spectrum, aiming to describe the heterogeneity of Guillain-Barr{\'e} syndrome worldwide. The current study was based on the first 1000 inclusions with a follow-up of at least 1 year and confirmed the variation in clinical presentation, course and outcome between patients. The full clinical spectrum of Guillain-Barr{\'e} syndrome was observed in patients from all countries participating in the International Guillain-Barr{\'e} Syndrome Outcome Study, but the frequency of variants differed between regions. We compared three regions based on geography, income and previous reports of Guillain-Barr{\'e} syndrome subtypes: Europe/Americas', Asia' (without Bangladesh), and Bangladesh'. We excluded 75 (8{\%}) patients because of alternative diagnoses, protocol violations, or missing data. The predominant clinical variant was sensorimotor in Europe/Americas (n = 387/562, 69{\%}) and Asia (n = 27/63, 43{\%}), and pure motor in Bangladesh (n = 74/107, 69{\%}). Miller Fisher syndrome and Miller Fisher-Guillain-Barr{\'e} overlap syndrome were more common in Asia (n = 14/63, 22{\%}) than in the other two regions (Europe/Americas: n = 64/562, 11{\%}; Bangladesh: n = 1/107, 1{\%}) (P < 0.001). The predominant electrophysiological subtype was demyelinating in all regions (Europe/Americas: n = 312/573, 55{\%}; Asia: n = 29/65, 45{\%}; Bangladesh: n = 38/94, 40{\%}). The axonal subtype occurred more often in Bangladesh (n = 34/94, 36{\%}) than in Europe/Americas (n = 33/573, 6{\%}) and other Asian countries (n = 4/65, 6{\%}) (P < 0.001). In all regions, patients with the axonal subtype were younger, had fewer sensory deficits, and showed a trend towards poorer recovery compared to patients with the demyelinating subtype. The proportion of patients able to walk unaided after 1 year varied between Asia (n = 31/34, 91{\%}), Europe/Americas (n = 334/404, 83{\%}) and Bangladesh (n = 67/97, 69{\%}) (P = 0.003). A similar variation was seen for mortality, being higher in Bangladesh (n = 19/114, 17{\%}) than in Europe/Americas (n = 23/486, 5{\%}) and Asia (n = 1/45, 2{\%}) (P < 0.001). This study showed that factors related to geography have a major influence on clinical phenotype, disease severity, electrophysiological subtype, and outcome of Guillain-Barr{\'e} syndrome.",
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T1 - Regional variation of Guillain-Barré syndrome

AU - Doets, Alex Y.

AU - Verboon, Christine

AU - Van Den Berg, Bianca

AU - Harbo, Thomas

AU - Cornblath, David R.

AU - Willison, Hugh J.

AU - Islam, Zhahirul

AU - Attarian, Shahram

AU - Barroso, Fabio A.

AU - Bateman, Kathleen

AU - Benedetti, Luana

AU - Van Den Bergh, Peter

AU - Casasnovas, Carlos

AU - Cavaletti, Guido

AU - Chavada, Govindsinh

AU - Claeys, Kristl G.

AU - Dardiotis, Efthimios

AU - Davidson, Amy

AU - Van Doorn, Pieter A.

AU - Feasby, Tom E.

AU - Galassi, Giuliana

AU - Gorson, Kenneth C.

AU - Hartung, Hans Peter

AU - Hsieh, Sung Tsang

AU - Hughes, Richard A.C.

AU - Illa, Isabel

AU - Islam, Badrul

AU - Kusunoki, Susumu

AU - Kuwabara, Satoshi

AU - Lehmann, Helmar C.

AU - Miller, James A.L.

AU - Mohammad, Quazi Deen

AU - Monges, Soledad

AU - Nobile Orazio, Eduardo

AU - Pardo, Julio

AU - Pereon, Yann

AU - Rinaldi, Simon

AU - Querol, Luis

AU - Reddel, Stephen W.

AU - Reisin, Ricardo C.

AU - Shahrizaila, Nortina

AU - Sindrup, Soren H.

AU - Waqar, Waheed

AU - Jacobs, Bart C.

PY - 2018/10/1

Y1 - 2018/10/1

N2 - Guillain-Barré syndrome is a heterogeneous disorder regarding the clinical presentation, electrophysiological subtype and outcome. Previous single country reports indicate that Guillain-Barré syndrome may differ among regions, but no systematic comparative studies have been conducted. Comparative studies are required to identify factors determining disease susceptibility, variation and prognosis, and to improve diagnostic criteria. The International Guillain-Barré Syndrome Outcome Study is a prospective, observational cohort study including all patients within the diagnostic spectrum, aiming to describe the heterogeneity of Guillain-Barré syndrome worldwide. The current study was based on the first 1000 inclusions with a follow-up of at least 1 year and confirmed the variation in clinical presentation, course and outcome between patients. The full clinical spectrum of Guillain-Barré syndrome was observed in patients from all countries participating in the International Guillain-Barré Syndrome Outcome Study, but the frequency of variants differed between regions. We compared three regions based on geography, income and previous reports of Guillain-Barré syndrome subtypes: Europe/Americas', Asia' (without Bangladesh), and Bangladesh'. We excluded 75 (8%) patients because of alternative diagnoses, protocol violations, or missing data. The predominant clinical variant was sensorimotor in Europe/Americas (n = 387/562, 69%) and Asia (n = 27/63, 43%), and pure motor in Bangladesh (n = 74/107, 69%). Miller Fisher syndrome and Miller Fisher-Guillain-Barré overlap syndrome were more common in Asia (n = 14/63, 22%) than in the other two regions (Europe/Americas: n = 64/562, 11%; Bangladesh: n = 1/107, 1%) (P < 0.001). The predominant electrophysiological subtype was demyelinating in all regions (Europe/Americas: n = 312/573, 55%; Asia: n = 29/65, 45%; Bangladesh: n = 38/94, 40%). The axonal subtype occurred more often in Bangladesh (n = 34/94, 36%) than in Europe/Americas (n = 33/573, 6%) and other Asian countries (n = 4/65, 6%) (P < 0.001). In all regions, patients with the axonal subtype were younger, had fewer sensory deficits, and showed a trend towards poorer recovery compared to patients with the demyelinating subtype. The proportion of patients able to walk unaided after 1 year varied between Asia (n = 31/34, 91%), Europe/Americas (n = 334/404, 83%) and Bangladesh (n = 67/97, 69%) (P = 0.003). A similar variation was seen for mortality, being higher in Bangladesh (n = 19/114, 17%) than in Europe/Americas (n = 23/486, 5%) and Asia (n = 1/45, 2%) (P < 0.001). This study showed that factors related to geography have a major influence on clinical phenotype, disease severity, electrophysiological subtype, and outcome of Guillain-Barré syndrome.

AB - Guillain-Barré syndrome is a heterogeneous disorder regarding the clinical presentation, electrophysiological subtype and outcome. Previous single country reports indicate that Guillain-Barré syndrome may differ among regions, but no systematic comparative studies have been conducted. Comparative studies are required to identify factors determining disease susceptibility, variation and prognosis, and to improve diagnostic criteria. The International Guillain-Barré Syndrome Outcome Study is a prospective, observational cohort study including all patients within the diagnostic spectrum, aiming to describe the heterogeneity of Guillain-Barré syndrome worldwide. The current study was based on the first 1000 inclusions with a follow-up of at least 1 year and confirmed the variation in clinical presentation, course and outcome between patients. The full clinical spectrum of Guillain-Barré syndrome was observed in patients from all countries participating in the International Guillain-Barré Syndrome Outcome Study, but the frequency of variants differed between regions. We compared three regions based on geography, income and previous reports of Guillain-Barré syndrome subtypes: Europe/Americas', Asia' (without Bangladesh), and Bangladesh'. We excluded 75 (8%) patients because of alternative diagnoses, protocol violations, or missing data. The predominant clinical variant was sensorimotor in Europe/Americas (n = 387/562, 69%) and Asia (n = 27/63, 43%), and pure motor in Bangladesh (n = 74/107, 69%). Miller Fisher syndrome and Miller Fisher-Guillain-Barré overlap syndrome were more common in Asia (n = 14/63, 22%) than in the other two regions (Europe/Americas: n = 64/562, 11%; Bangladesh: n = 1/107, 1%) (P < 0.001). The predominant electrophysiological subtype was demyelinating in all regions (Europe/Americas: n = 312/573, 55%; Asia: n = 29/65, 45%; Bangladesh: n = 38/94, 40%). The axonal subtype occurred more often in Bangladesh (n = 34/94, 36%) than in Europe/Americas (n = 33/573, 6%) and other Asian countries (n = 4/65, 6%) (P < 0.001). In all regions, patients with the axonal subtype were younger, had fewer sensory deficits, and showed a trend towards poorer recovery compared to patients with the demyelinating subtype. The proportion of patients able to walk unaided after 1 year varied between Asia (n = 31/34, 91%), Europe/Americas (n = 334/404, 83%) and Bangladesh (n = 67/97, 69%) (P = 0.003). A similar variation was seen for mortality, being higher in Bangladesh (n = 19/114, 17%) than in Europe/Americas (n = 23/486, 5%) and Asia (n = 1/45, 2%) (P < 0.001). This study showed that factors related to geography have a major influence on clinical phenotype, disease severity, electrophysiological subtype, and outcome of Guillain-Barré syndrome.

KW - axonal degeneration

KW - clinical course

KW - demyelination

KW - outcome

KW - polyradiculoneuropathy

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