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