John P Osborne1,2, Stuart W Edwards1,2, Fabienne Dietrich Alber3, Eleanor Hancock2, Anthony L Johnson4, Colin R Kennedy5, Marcus Likeman6, Andrew L Lux7, Mark Mackay8, Andrew Mallick7, Richard W Newton9, Melinda Nolan10, Ronit Pressler11, Dietz Rating12, Bernhard Schmitt13, Christopher M Verity14, Finbar J K O'Callaghan2,15. 1. Department for Health, University of Bath, Bath, UK. 2. Children's Department, Royal United Hospitals Bath NHS Foundation Trust, Bath, UK. 3. Division of Neurology/Neuropsychology, University Children's Hospital, Zurich, Switzerland. 4. Medical Research Council Clinical Trials Unit at UCL, Institute of Clinical Trials and Methodology, London, UK. 5. Clinical Neurosciences, Faculty of Medicine, University of Southampton, Southampton General Hospital, Southampton, UK. 6. Department of Paediatric Radiology, Bristol Royal Hospital for Children, Bristol, UK. 7. Department of Paediatric Neurology, Bristol Royal Hospital for Children, Bristol, UK. 8. Neurology Department, The Royal Children's Hospital Melbourne, Parkville, Vic., Australia. 9. Department of Neurology, Royal Manchester Children's Hospital, Manchester, UK. 10. Starship Children's Health, Auckland, New Zealand. 11. UCL Institute of Child Health, Clinical Neurosciences, London, UK. 12. University of Heidelberg, Heidelberg, Germany. 13. Division of Paediatric Neurology, University Children's Hospital, Zurich, Switzerland. 14. PIND research, Addenbrookes Hospital, Cambridge, UK. 15. Institute of Child Health, University College London, London, UK.
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. Wiley Periodicals, Inc.
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. Wiley Periodicals, Inc.
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