Elysa Widjaja1, Cristina Go2, Blathnaid McCoy3, O Carter Snead4. 1. Diagnostic Imaging, The Hospital for Sick Children, Toronto, Ontario, Canada; Division of Neurology, The Hospital for Sick Children, Toronto, Ontario, Canada. Electronic address: Elysa.Widjaja@sickkids.ca. 2. Division of Neurology, The Hospital for Sick Children, Toronto, Ontario, Canada. Electronic address: Cristina.Go@sickkids.ca. 3. Division of Neurology, The Hospital for Sick Children, Toronto, Ontario, Canada. Electronic address: Blathnaid.McCoy@sickkids.ca. 4. Division of Neurology, The Hospital for Sick Children, Toronto, Ontario, Canada. Electronic address: Carter.Snead@sickkids.ca.
Abstract
BACKGROUND: The aims of this systematic review and meta-analysis were to assess (i) estimates of good neurodevelopmental outcome in infantile spasms (IS), (ii) if neurodevelopmental outcome has changed since the publication of the first guideline on medical treatment of IS in 2004 and (iii) effect of lead time to treatment (LTTT). METHODS: The Medline, Embase, Cochrane, PsycINFO, Web of Science and Scopus databases, and reference lists of retrieved articles were searched. Studies inclusion criteria were: (i) >5 patients with IS, (ii) mean/median follow-up of >6 months, (iii) neurodevelopmental outcome, and (iv) randomized and observational studies. The data extracted included proportion of good neurodevelopmental outcome, year of publication, cryptogenic or symptomatic IS and LTTT. RESULTS: Of the 1436 citations screened, 55 articles were included in final analysis, with a total of 2967 patients. The pooled estimate for good neurodevelopmental outcome was 0.236 (95% CI: 0.193-0.286). There was no difference between the proportions of good neurodevelopmental outcome for the 21 studies published after 2004 [0.264 (95% CI: 0.197-0.344)] compared to the 34 studies published before 2004 [0.220 (95% CI: 0.168-0.283)] (Q value=0.862, p=0.353). The pooled estimate of good neurodevelopmental outcome for cryptogenic IS [0.543 (95% CI: 0.458-0.625)] was higher than symptomatic IS [0.125 (95% CI: 0.09-0.171)] (Q value=69.724, p<0.001). Risk ratio of LTTT <4weeks relative to >4weeks for good neurodevelopmental outcome of 8 studies was 1.519 (95% CI: 1.064-2.169). CONCLUSION: Neurodevelopmental outcome was overall poor in patients with IS and has not changed since the publication of first guideline on IS. Although cryptogenic IS has better prognosis than symptomatic IS, the outcome for cryptogenic IS remained poor. There was heterogeneity in neurodevelopmental outcome ascertainment methods, highlighting the need for a more standardized and comprehensive assessment of cognitive, behavioural, emotional and functional outcomes.
BACKGROUND: The aims of this systematic review and meta-analysis were to assess (i) estimates of good neurodevelopmental outcome in infantile spasms (IS), (ii) if neurodevelopmental outcome has changed since the publication of the first guideline on medical treatment of IS in 2004 and (iii) effect of lead time to treatment (LTTT). METHODS: The Medline, Embase, Cochrane, PsycINFO, Web of Science and Scopus databases, and reference lists of retrieved articles were searched. Studies inclusion criteria were: (i) >5 patients with IS, (ii) mean/median follow-up of >6 months, (iii) neurodevelopmental outcome, and (iv) randomized and observational studies. The data extracted included proportion of good neurodevelopmental outcome, year of publication, cryptogenic or symptomatic IS and LTTT. RESULTS: Of the 1436 citations screened, 55 articles were included in final analysis, with a total of 2967 patients. The pooled estimate for good neurodevelopmental outcome was 0.236 (95% CI: 0.193-0.286). There was no difference between the proportions of good neurodevelopmental outcome for the 21 studies published after 2004 [0.264 (95% CI: 0.197-0.344)] compared to the 34 studies published before 2004 [0.220 (95% CI: 0.168-0.283)] (Q value=0.862, p=0.353). The pooled estimate of good neurodevelopmental outcome for cryptogenic IS [0.543 (95% CI: 0.458-0.625)] was higher than symptomatic IS [0.125 (95% CI: 0.09-0.171)] (Q value=69.724, p<0.001). Risk ratio of LTTT <4weeks relative to >4weeks for good neurodevelopmental outcome of 8 studies was 1.519 (95% CI: 1.064-2.169). CONCLUSION: Neurodevelopmental outcome was overall poor in patients with IS and has not changed since the publication of first guideline on IS. Although cryptogenic IS has better prognosis than symptomatic IS, the outcome for cryptogenic IS remained poor. There was heterogeneity in neurodevelopmental outcome ascertainment methods, highlighting the need for a more standardized and comprehensive assessment of cognitive, behavioural, emotional and functional outcomes.
Authors: Kelly G Knupp; Erin Leister; Jason Coryell; Katherine C Nickels; Nicole Ryan; Elizabeth Juarez-Colunga; William D Gaillard; John R Mytinger; Anne T Berg; John Millichap; Douglas R Nordli; Sucheta Joshi; Renée A Shellhaas; Tobias Loddenkemper; Dennis Dlugos; Elaine Wirrell; Joseph Sullivan; Adam L Hartman; Eric H Kossoff; Zachary M Grinspan; Lorie Hamikawa Journal: Epilepsia Date: 2016-09-12 Impact factor: 5.864
Authors: Charlotte DiStefano; Rujuta B Wilson; Carly Hyde; Edwin H Cook; Ronald L Thibert; Lawrence T Reiter; Vanessa Vogel-Farley; Joerg Hipp; Shafali Jeste Journal: Am J Med Genet A Date: 2019-10-26 Impact factor: 2.802