Natural history of benign multiple sclerosis: Clinical and HLA correlates in a Western Australian cohort

Marzena J. Fabis-Pedrini, Ian James, Alexandra Seewann, Wai Y. Yau, Arnold A. van de Bovenkamp, Fay R.K. Sanders, Wei Qiu, Jason Burton, Frank L. Mastaglia, William M. Carroll, Allan G. Kermode

Research output: Contribution to journalArticle

Abstract

Background: Benign multiple sclerosis (BMS) is a controversial term that has been used for MS patients with minimal disability decades after disease onset. Herein, we evaluated disease status after 20 years in a Western Australian cohort defined as BMS based on an Expanded Disability Status Scale (EDSS) score ≤ 3.0 at 10 years from onset. Methods: MS patients with an EDSS score ≤ 3.0 at 10 years from onset and minimum of 20 years follow up were included in the study. The 20-year EDSS score was considered the primary outcome. Associations with demographic and clinical characteristics and HLA-DRB1 genotype were investigated. Results: Among 120 patients with a benign course at 10 years, 78 (65%) remained benign at the 20-year follow up, but patients with an EDSS ≥ 2.5 were more likely to go on to develop more severe disability in the next decade. When considering factors associated with an increase in EDSS score ≤ 1 from 10 to 20 years, indicating limited progression, apart from the EDSS score at 10 years, poly-symptomatic presentation (p = 0.004) and cerebellar/brainstem mono-symptomatic presentation (p = 0.016) were independently associated with more rapid progression compared with other mono-symptomatic presentations. Carriage of the high risk HLA-DRB1*1501 allele was marginally associated with slower progression. Conclusions: In this geographically isolated MS cohort of predominantly Anglo-Celtic origin clinical progression in the benign MS group was similar to that in other published series from Western countries. These results are in keeping with the view that patients labeled as benign MS are part of a heterogeneous continuum of disease progression and do not possess unique clinical characteristics. Possible genetic determinants of a benign course warrant further investigation.

Original languageEnglish
Pages (from-to)12-18
Number of pages7
JournalJournal of the Neurological Sciences
Volume388
DOIs
Publication statusPublished - 15 May 2018

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Natural History
Multiple Sclerosis
HLA-DRB1 Chains
Brain Stem
Disease Progression
Alleles
Genotype
Demography

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Fabis-Pedrini, Marzena J. ; James, Ian ; Seewann, Alexandra ; Yau, Wai Y. ; van de Bovenkamp, Arnold A. ; Sanders, Fay R.K. ; Qiu, Wei ; Burton, Jason ; Mastaglia, Frank L. ; Carroll, William M. ; Kermode, Allan G. / Natural history of benign multiple sclerosis : Clinical and HLA correlates in a Western Australian cohort. In: Journal of the Neurological Sciences. 2018 ; Vol. 388. pp. 12-18.
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title = "Natural history of benign multiple sclerosis: Clinical and HLA correlates in a Western Australian cohort",
abstract = "Background: Benign multiple sclerosis (BMS) is a controversial term that has been used for MS patients with minimal disability decades after disease onset. Herein, we evaluated disease status after 20 years in a Western Australian cohort defined as BMS based on an Expanded Disability Status Scale (EDSS) score ≤ 3.0 at 10 years from onset. Methods: MS patients with an EDSS score ≤ 3.0 at 10 years from onset and minimum of 20 years follow up were included in the study. The 20-year EDSS score was considered the primary outcome. Associations with demographic and clinical characteristics and HLA-DRB1 genotype were investigated. Results: Among 120 patients with a benign course at 10 years, 78 (65{\%}) remained benign at the 20-year follow up, but patients with an EDSS ≥ 2.5 were more likely to go on to develop more severe disability in the next decade. When considering factors associated with an increase in EDSS score ≤ 1 from 10 to 20 years, indicating limited progression, apart from the EDSS score at 10 years, poly-symptomatic presentation (p = 0.004) and cerebellar/brainstem mono-symptomatic presentation (p = 0.016) were independently associated with more rapid progression compared with other mono-symptomatic presentations. Carriage of the high risk HLA-DRB1*1501 allele was marginally associated with slower progression. Conclusions: In this geographically isolated MS cohort of predominantly Anglo-Celtic origin clinical progression in the benign MS group was similar to that in other published series from Western countries. These results are in keeping with the view that patients labeled as benign MS are part of a heterogeneous continuum of disease progression and do not possess unique clinical characteristics. Possible genetic determinants of a benign course warrant further investigation.",
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Natural history of benign multiple sclerosis : Clinical and HLA correlates in a Western Australian cohort. / Fabis-Pedrini, Marzena J.; James, Ian; Seewann, Alexandra; Yau, Wai Y.; van de Bovenkamp, Arnold A.; Sanders, Fay R.K.; Qiu, Wei; Burton, Jason; Mastaglia, Frank L.; Carroll, William M.; Kermode, Allan G.

In: Journal of the Neurological Sciences, Vol. 388, 15.05.2018, p. 12-18.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Natural history of benign multiple sclerosis

T2 - Clinical and HLA correlates in a Western Australian cohort

AU - Fabis-Pedrini, Marzena J.

AU - James, Ian

AU - Seewann, Alexandra

AU - Yau, Wai Y.

AU - van de Bovenkamp, Arnold A.

AU - Sanders, Fay R.K.

AU - Qiu, Wei

AU - Burton, Jason

AU - Mastaglia, Frank L.

AU - Carroll, William M.

AU - Kermode, Allan G.

PY - 2018/5/15

Y1 - 2018/5/15

N2 - Background: Benign multiple sclerosis (BMS) is a controversial term that has been used for MS patients with minimal disability decades after disease onset. Herein, we evaluated disease status after 20 years in a Western Australian cohort defined as BMS based on an Expanded Disability Status Scale (EDSS) score ≤ 3.0 at 10 years from onset. Methods: MS patients with an EDSS score ≤ 3.0 at 10 years from onset and minimum of 20 years follow up were included in the study. The 20-year EDSS score was considered the primary outcome. Associations with demographic and clinical characteristics and HLA-DRB1 genotype were investigated. Results: Among 120 patients with a benign course at 10 years, 78 (65%) remained benign at the 20-year follow up, but patients with an EDSS ≥ 2.5 were more likely to go on to develop more severe disability in the next decade. When considering factors associated with an increase in EDSS score ≤ 1 from 10 to 20 years, indicating limited progression, apart from the EDSS score at 10 years, poly-symptomatic presentation (p = 0.004) and cerebellar/brainstem mono-symptomatic presentation (p = 0.016) were independently associated with more rapid progression compared with other mono-symptomatic presentations. Carriage of the high risk HLA-DRB1*1501 allele was marginally associated with slower progression. Conclusions: In this geographically isolated MS cohort of predominantly Anglo-Celtic origin clinical progression in the benign MS group was similar to that in other published series from Western countries. These results are in keeping with the view that patients labeled as benign MS are part of a heterogeneous continuum of disease progression and do not possess unique clinical characteristics. Possible genetic determinants of a benign course warrant further investigation.

AB - Background: Benign multiple sclerosis (BMS) is a controversial term that has been used for MS patients with minimal disability decades after disease onset. Herein, we evaluated disease status after 20 years in a Western Australian cohort defined as BMS based on an Expanded Disability Status Scale (EDSS) score ≤ 3.0 at 10 years from onset. Methods: MS patients with an EDSS score ≤ 3.0 at 10 years from onset and minimum of 20 years follow up were included in the study. The 20-year EDSS score was considered the primary outcome. Associations with demographic and clinical characteristics and HLA-DRB1 genotype were investigated. Results: Among 120 patients with a benign course at 10 years, 78 (65%) remained benign at the 20-year follow up, but patients with an EDSS ≥ 2.5 were more likely to go on to develop more severe disability in the next decade. When considering factors associated with an increase in EDSS score ≤ 1 from 10 to 20 years, indicating limited progression, apart from the EDSS score at 10 years, poly-symptomatic presentation (p = 0.004) and cerebellar/brainstem mono-symptomatic presentation (p = 0.016) were independently associated with more rapid progression compared with other mono-symptomatic presentations. Carriage of the high risk HLA-DRB1*1501 allele was marginally associated with slower progression. Conclusions: In this geographically isolated MS cohort of predominantly Anglo-Celtic origin clinical progression in the benign MS group was similar to that in other published series from Western countries. These results are in keeping with the view that patients labeled as benign MS are part of a heterogeneous continuum of disease progression and do not possess unique clinical characteristics. Possible genetic determinants of a benign course warrant further investigation.

KW - Benign

KW - Disability

KW - Long term progression

KW - Multiple sclerosis

KW - Prognostic factors

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