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The underlying aetiology of infantile spasms (West Syndrome): Information from the International Collaborative Infantile Spasms Study (ICISS)

The underlying aetiology of infantile spasms (West Syndrome): Information from the International Collaborative Infantile Spasms Study (ICISS)
The underlying aetiology of infantile spasms (West Syndrome): Information from the International Collaborative Infantile Spasms Study (ICISS)
Objective
To determine the underlying etiologies in a contemporary cohort of infants with infantile spasms and to examine response to treatment.

Methods
Identification of the underlying etiology and response to treatment in 377 infants enrolled in a clinical trial of the treatment of infantile spasms between 2007 and 2014 using a systematic review of history, examination, and investigations. They were classified using the pediatric adaptation of International Classification of Diseases, Tenth Revision (ICD‐10).

Results
A total of 219 of 377 (58%) had a proven etiology, of whom 128 (58%) responded, 58 of 108 (54%) were allocated hormonal treatment, and 70 of 111 (63%) had combination therapy. Fourteen of 17 (82%, 95% confidence interval [CI] 59% to 94%) infants with stroke and infarct responded (compared to 114 of 202 for the rest of the proven etiology group (56%, 95% CI 48% to 62%, chi‐square 4.3, P = .037): the better response remains when treatment allocation and lead time are taken into account (odds ratio 5.1, 95% CI 1.1 to 23.6, P = .037). Twenty of 37 (54%, 95% CI 38% to 70%) infants with Down syndrome had cessation of spasms compared to 108 of 182 (59%, 95% CI 52% to 66%, chi‐square 0.35, P = .55) for the rest of the proven etiology group. The lack of a significant difference remains after taking treatment modality and lead‐time into account (odds ratio 0.8, 95% CI 0.4 to 1.7, P = .62). In Down syndrome infants, treatment modality did not appear to affect response: 11 of 20 (55%) allocated hormonal therapy responded, compared to 9 of 17 (53%) allocated combination therapy.

Significance
This classification allows easy comparison with other classifications and with our earlier reports. Stroke and infarct have a better outcome than other etiologies, whereas Down syndrome might not respond to the addition of vigabatrin to hormonal treatment.
0013-9580
1861-1869
Osborne, John P.
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Edwards, Stuart W.
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Dietrich Alber, Fabienne
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Hancock, Eleanor
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Johnson, Anthony L.
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Kennedy, Colin
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Likeman, Marcus
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Lux, Andrew L.
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Mackay, Mark
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Mallick, Andrew A.
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Newton, Richard W.
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Nolan, Melinda
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Pressler, Ronit
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Rating, Dietz
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Schmitt, Bernhard
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Verity, Christopher M.
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O'Callaghan, Finbar J.K.
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Osborne, John P.
db90ab19-3de3-49a8-b82a-b050a1180e6f
Edwards, Stuart W.
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Dietrich Alber, Fabienne
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Hancock, Eleanor
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Johnson, Anthony L.
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Kennedy, Colin
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Likeman, Marcus
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Lux, Andrew L.
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Mackay, Mark
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Mallick, Andrew A.
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Newton, Richard W.
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Nolan, Melinda
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Pressler, Ronit
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Rating, Dietz
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Schmitt, Bernhard
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Verity, Christopher M.
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O'Callaghan, Finbar J.K.
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Osborne, John P., Edwards, Stuart W., Dietrich Alber, Fabienne, Hancock, Eleanor, Johnson, Anthony L., Kennedy, Colin, Likeman, Marcus, Lux, Andrew L., Mackay, Mark, Mallick, Andrew A., Newton, Richard W., Nolan, Melinda, Pressler, Ronit, Rating, Dietz, Schmitt, Bernhard, Verity, Christopher M. and O'Callaghan, Finbar J.K. (2019) The underlying aetiology of infantile spasms (West Syndrome): Information from the International Collaborative Infantile Spasms Study (ICISS). Epilepsia, 60 (9), 1861-1869. (doi:10.1111/epi.16305).

Record type: Article

Abstract

Objective
To determine the underlying etiologies in a contemporary cohort of infants with infantile spasms and to examine response to treatment.

Methods
Identification of the underlying etiology and response to treatment in 377 infants enrolled in a clinical trial of the treatment of infantile spasms between 2007 and 2014 using a systematic review of history, examination, and investigations. They were classified using the pediatric adaptation of International Classification of Diseases, Tenth Revision (ICD‐10).

Results
A total of 219 of 377 (58%) had a proven etiology, of whom 128 (58%) responded, 58 of 108 (54%) were allocated hormonal treatment, and 70 of 111 (63%) had combination therapy. Fourteen of 17 (82%, 95% confidence interval [CI] 59% to 94%) infants with stroke and infarct responded (compared to 114 of 202 for the rest of the proven etiology group (56%, 95% CI 48% to 62%, chi‐square 4.3, P = .037): the better response remains when treatment allocation and lead time are taken into account (odds ratio 5.1, 95% CI 1.1 to 23.6, P = .037). Twenty of 37 (54%, 95% CI 38% to 70%) infants with Down syndrome had cessation of spasms compared to 108 of 182 (59%, 95% CI 52% to 66%, chi‐square 0.35, P = .55) for the rest of the proven etiology group. The lack of a significant difference remains after taking treatment modality and lead‐time into account (odds ratio 0.8, 95% CI 0.4 to 1.7, P = .62). In Down syndrome infants, treatment modality did not appear to affect response: 11 of 20 (55%) allocated hormonal therapy responded, compared to 9 of 17 (53%) allocated combination therapy.

Significance
This classification allows easy comparison with other classifications and with our earlier reports. Stroke and infarct have a better outcome than other etiologies, whereas Down syndrome might not respond to the addition of vigabatrin to hormonal treatment.

Text
Osborne et al UKISS-ICISS aetiology Epilepsia manuscript revised clean 20June19 - Accepted Manuscript
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More information

Accepted/In Press date: 9 July 2019
e-pub ahead of print date: 16 August 2019
Published date: September 2019

Identifiers

Local EPrints ID: 433910
URI: http://eprints.soton.ac.uk/id/eprint/433910
ISSN: 0013-9580
PURE UUID: a99e033c-13ad-4719-ba1e-b6e6f21116c8

Catalogue record

Date deposited: 06 Sep 2019 16:30
Last modified: 26 Nov 2021 06:12

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Contributors

Author: John P. Osborne
Author: Stuart W. Edwards
Author: Fabienne Dietrich Alber
Author: Eleanor Hancock
Author: Anthony L. Johnson
Author: Colin Kennedy
Author: Marcus Likeman
Author: Andrew L. Lux
Author: Mark Mackay
Author: Andrew A. Mallick
Author: Richard W. Newton
Author: Melinda Nolan
Author: Ronit Pressler
Author: Dietz Rating
Author: Bernhard Schmitt
Author: Christopher M. Verity
Author: Finbar J.K. O'Callaghan

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