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The underlying etiology of infantile spasms (West syndrome): Information from the International Collaborative Infantile Spasms Study (ICISS)
- 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
-
Abstract
- ObjectiveTo determine the underlying etiologies in a contemporary cohort of infants with infantile spasms and to examine response to treatment.MethodsIdentification 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).ResultsA 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.SignificanceThis 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.
- Subjects :
- Male
0301 basic medicine
Down syndrome
Pediatrics
medicine.medical_specialty
Combination therapy
Prednisolone
etiology
Clinical Neurology
Vigabatrin
03 medical and health sciences
0302 clinical medicine
Humans
Medicine
Stroke
business.industry
Infant
Odds ratio
West syndrome
medicine.disease
Confidence interval
Malformations of Cortical Development
030104 developmental biology
Neurology
Cohort
Etiology
Anticonvulsants
Female
Neurology (clinical)
business
Spasms, Infantile
030217 neurology & neurosurgery
medicine.drug
infantile spasms
Subjects
Details
- Language :
- English
- Database :
- OpenAIRE
- Journal :
- 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
- Accession number :
- edsair.doi.dedup.....6fe2e2ca93c7f37482cebcac0efbd694