The underlying etiology of infantile spasms (West syndrome): Information from the International Collaborative Infantile Spasms Study (ICISS).
- Osborne JP Department for Health, University of Bath, Bath, UK.
- Edwards SW Department for Health, University of Bath, Bath, UK.
- Dietrich Alber F Division of Neurology/Neuropsychology, University Children's Hospital, Zurich, Switzerland.
- Hancock E Children's Department, Royal United Hospitals Bath NHS Foundation Trust, Bath, UK.
- Johnson AL Medical Research Council Clinical Trials Unit at UCL, Institute of Clinical Trials and Methodology, London, UK.
- Kennedy CR Clinical Neurosciences, Faculty of Medicine, University of Southampton, Southampton General Hospital, Southampton, UK.
- Likeman M Department of Paediatric Radiology, Bristol Royal Hospital for Children, Bristol, UK.
- Lux AL Department of Paediatric Neurology, Bristol Royal Hospital for Children, Bristol, UK.
- Mackay M Neurology Department, The Royal Children's Hospital Melbourne, Parkville, Vic., Australia.
- Mallick A Department of Paediatric Neurology, Bristol Royal Hospital for Children, Bristol, UK.
- Newton RW Department of Neurology, Royal Manchester Children's Hospital, Manchester, UK.
- Nolan M Starship Children's Health, Auckland, New Zealand.
- Pressler R UCL Institute of Child Health, Clinical Neurosciences, London, UK.
- Rating D University of Heidelberg, Heidelberg, Germany.
- Schmitt B Division of Paediatric Neurology, University Children's Hospital, Zurich, Switzerland.
- Verity CM PIND research, Addenbrookes Hospital, Cambridge, UK.
- O'Callaghan FJK Children's Department, Royal United Hospitals Bath NHS Foundation Trust, Bath, UK.
- 2019-08-17
Published in:
- Epilepsia. - 2019
West syndrome
etiology
infantile spasms
Anticonvulsants
Female
Humans
Infant
Male
Malformations of Cortical Development
Prednisolone
Spasms, Infantile
Stroke
Vigabatrin
English
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.
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.
- Language
-
- English
- Open access status
- bronze
- Identifiers
-
- DOI 10.1111/epi.16305
- PMID 31418851
- Persistent URL
- https://sonar.ch/global/documents/235739
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