| Title: |
The underlying etiology of infantile spasms (West syndrome): Information from the International Collaborative Infantile Spasms Study (ICISS) |
| Authors: |
Osborne, John P.; Edwards, Stuart W.; Dietrich Alber, Fabienne; Hancock, Eleanor; Johnson, Anthony L.; Kennedy, Colin R.; Likeman, Marcus; Lux, Andrew L.; Mackay, Mark; Mallick, Andrew; Newton, Richard W.; Nolan, Melinda; Pressler, Ronit; Rating, Dietz; Schmitt, Bernhard; Verity, Christopher M.; O'Callaghan, Finbar J. K.; on behalf of the participating investigators |
| Source: |
Osborne, J P, Edwards, S W, Dietrich Alber, F, Hancock, E, Johnson, A L, Kennedy, C R, Likeman, M, Lux, A L, Mackay, M, Mallick, A, Newton, R W, Nolan, M, Pressler, R, Rating, D, Schmitt, B, Verity, C M, O'Callaghan, F J K & on behalf of the participating investigators 2019, 'The underlying etiology of infantile spasms (West syndrome): Information from the International Collaborative Infantile Spasms Study (ICISS)', Epilepsia, vol. 60, no. 9, pp. 1861-1869. https://doi.org/10.1111/epi.16305 |
| Publication Year: |
2019 |
| Subject Terms: |
West syndrome; etiology; infantile spasms; /dk/atira/pure/subjectarea/asjc/2800/2808; name=Neurology; /dk/atira/pure/subjectarea/asjc/2700/2728; name=Clinical Neurology |
| Description: |
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. |
| Document Type: |
article in journal/newspaper |
| File Description: |
application/pdf |
| Language: |
English |
| DOI: |
10.1111/epi.16305 |
| Availability: |
https://researchportal.bath.ac.uk/en/publications/7c25d477-dcd4-4f59-a643-0bc984fe7ec7; https://doi.org/10.1111/epi.16305; https://purehost.bath.ac.uk/ws/files/199142881/Osborne_manuscript_09.7.19_accepted_version_with_link_locked_.pdf; http://www.scopus.com/inward/record.url?scp=85070764049&partnerID=8YFLogxK |
| Rights: |
info:eu-repo/semantics/openAccess |
| Accession Number: |
edsbas.16592DCB |
| Database: |
BASE |