Response to treatment in a prospective national infantile spasms cohort
Kelly G. Knupp,Jason Coryell,Katherine C. Nickels,Nicole Ryan,Erin C. Leister,Tobias Loddenkemper,Zachary M. Grinspan,Adam L. Hartman,Eric H. Kossoff,William D. Gaillard,John R. Mytinger,Sucheta M. Joshi,Renée A. Shellhaas,Joseph Sullivan,Dennis J. Dlugos,Lorie Hamikawa,Anne T. Berg,John Millichap,Douglas R. Nordli,Elaine C. Wirrell +19 more
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TLDR
This study evaluates early and sustained response to initial treatments and addresses both clinical remission and electrographic resolution of hypsarrhythmia in infants with infantile spasms.Abstract:
Objective Infantile spasms are seizures associated with a severe epileptic encephalopathy presenting in the first 2 years of life, and optimal treatment continues to be debated. This study evaluates early and sustained response to initial treatments and addresses both clinical remission and electrographic resolution of hypsarrhythmia. Secondarily, it assesses whether response to treatment differs by etiology or developmental status. Methods The National Infantile Spasms Consortium established a multicenter, prospective database enrolling infants with new diagnosis of infantile spasms. Children were considered responders if there was clinical remission and resolution of hypsarrhythmia that was sustained at 3 months after first treatment initiation. Standard treatments of adrenocorticotropic hormone (ACTH), oral corticosteroids, and vigabatrin were considered individually, and all other nonstandard therapies were analyzed collectively. Developmental status and etiology were assessed. We compared response rates by treatment group using chi-square tests and multivariate logistic regression models. Results Two hundred thirty infants were enrolled from 22 centers. Overall, 46% of children receiving standard therapy responded, compared to only 9% who responded to nonstandard therapy (p Interpretation Response rate varies by treatment choice. Standard therapies should be considered as initial treatment for infantile spasms, including those with impaired development or known structural or genetic/metabolic etiology. ACTH appeared to be more effective than other standard therapies.read more
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Safety and effectiveness of hormonal treatment versus hormonal treatment with vigabatrin for infantile spasms (ICISS): a randomised, multicentre, open-label trial
Finbar O'Callaghan,Finbar O'Callaghan,Stuart W Edwards,Stuart W Edwards,Fabienne Dietrich Alber,Eleanor Hancock,Anthony L. Johnson,Colin R. Kennedy,Marcus Likeman,Andrew L Lux,Mark T Mackay,Andrew A Mallick,Richard W Newton,Melinda Nolan,Ronit M. Pressler,Dietz Rating,Bernhard Schmitt,Christopher M Verity,John P. Osborne,John P. Osborne +19 more
TL;DR: Hormonal therapy with vigabatrin is significantly more effective at stopping infantile spasms than hormonal therapy alone and the 4 week period of spasm cessation required to achieve a primary clinical response to treatment suggests that the effect seen might be sustained, but this needs to be confirmed at the 18 month follow-up.
Journal ArticleDOI
Cyclin-Dependent Kinase-Like 5 Deficiency Disorder: Clinical Review.
Heather E. Olson,Scott Demarest,Elia Pestana-Knight,Lindsay C. Swanson,Sumaiya Iqbal,Sumaiya Iqbal,Dennis Lal,Dennis Lal,Helen Leonard,J. Helen Cross,Orrin Devinsky,Tim A. Benke +11 more
TL;DR: The clinical presentations and genetic variations in CDD are reviewed based on a systematic literature review and experience in the CDKL5 Centers of Excellence, and a minimum diagnostic criteria is proposed.
Journal ArticleDOI
Response to second treatment after initial failed treatment in a multicenter prospective infantile spasms cohort.
Kelly G. Knupp,Erin C. 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 M. Joshi,Renée A. Shellhaas,Tobias Loddenkemper,Dennis J. Dlugos,Elaine C. Wirrell,Joseph Sullivan,Adam L. Hartman,Eric H. Kossoff,Zachary M. Grinspan,Lorie Hamikawa +20 more
TL;DR: This work evaluated response to second treatment for IS in children in whom the initial therapy failed to produce both clinical remission and electrographic resolution of hypsarhythmia and whether time to treatment was related to outcome.
Journal ArticleDOI
Melanocortin Regulation of Inflammation.
TL;DR: It is now known that ACTH, together with α-, β-, and γ-MSHs, also possess glucocorticoid-independent anti-inflammatory and immunomodulatory effects by activating the melanocortin receptors expressed in the brain or peripheral immune cells.
Journal ArticleDOI
Infantile Spasms: Outcome in Clinical Studies
TL;DR: This review is based mainly on prospective studies and emphasizes data about the current first-line drugs, adrenocorticotropic hormone, vigabatrin, and prednisolone, taking into account the proportion of patients with known and unknown etiology, which has a very strong effect on seizure outcome.
References
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Anne T. Berg,Anne T. Berg,Samuel F. Berkovic,Martin J. Brodie,Jeffrey Buchhalter,J. Helen Cross,Walter van Emde Boas,Jerome Engel,Jacqueline A. French,Tracy A. Glauser,Gary W. Mathern,Solomon L. Moshé,Douglas R. Nordli,Perrine Plouin,Ingrid E. Scheffer +14 more
TL;DR: The International League Against Epilepsy (ILAE) Commission on Classification and Terminology has revised concepts, terminology, and approaches for classifying seizures and forms of epilepsy.
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Geometric method for measuring body surface area: A height-weight formula validated in infants, children, and adults†
TL;DR: Estimates of body surface area were made based on measurement of 81 subjects, ranging from premature infants to adults, and closer agreement was obtained with the equations and nomograms of Body, Brody, Faber and Melcher, and Sendroy and Cecchini, although minor deviations were noted in some age ranges.
Journal ArticleDOI
Poor Predictive Validity of the Bayley Scales of Infant Development for Cognitive Function of Extremely Low Birth Weight Children at School Age
Maureen Hack,H. Gerry Taylor,Dennis Drotar,Mark D. Schluchter,Lydia Cartar,Deanne E. Wilson-Costello,Nancy Klein,Harriet Friedman,Nori Mercuri-Minich,Mary Morrow +9 more
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Journal ArticleDOI
A proposal for case definitions and outcome measures in studies of infantile spasms and West syndrome: consensus statement of the West Delphi group.
Andrew L Lux,John P. Osborne +1 more
TL;DR: To reach a broad consensus on case definitions, outcomes, and outcome measures that will ease future study design and facilitate comparison of data from different studies of infantile spasms and West syndrome, a meta-analysis is presented.
Journal ArticleDOI
A prospective study of infantile spasms: clinical and therapeutic correlations.
TL;DR: Of 363 infants with infantile spasms (IS) admitted into the authors' hospital during an 18‐year period, 286 were closely followed for at least 6 years and form the basis of this report.