Literature DB >> 25895911

Improving Treatment Trial Outcomes for Rett Syndrome: The Development of Rett-specific Anchors for the Clinical Global Impression Scale.

Jeffrey L Neul1, Daniel G Glaze2, Alan K Percy3, Tim Feyma4, Arthur Beisang4, Thuy Dinh5, Bernhard Suter5, Evdokia Anagnostou6, Mike Snape7, Joseph Horrigan8, Nancy E Jones8.   

Abstract

Rett syndrome is a genetically based neurodevelopmental disorder. Although the clinical consequences of Rett syndrome are profound and lifelong, currently no approved drug treatments are available specifically targeted to Rett symptoms. High quality outcome measures, specific to the core symptoms of a disorder are a critical component of well-designed clinical trials for individuals with neurodevelopmental disorders. The Clinical Global Impression Scale is a measure of global clinical change with strong face validity that has been widely used as an outcome measure in clinical trials of central nervous system disorders. Despite its favorable assay sensitivity in clinical trials, as a global measure, the Clinical Global Impression Scale is not specific to the signs and symptoms of the disorder under study. Development of key anchors for the scale, specific to the disorder being assessed, holds promise for enhancing the validity and reliability of the measure for disorders such as Rett syndrome.
© The Author(s) 2015.

Entities:  

Keywords:  Clinical Global Impression Scales; MECP2; Rett syndrome; clinical trials; outcome measures

Mesh:

Substances:

Year:  2015        PMID: 25895911      PMCID: PMC4610825          DOI: 10.1177/0883073815579707

Source DB:  PubMed          Journal:  J Child Neurol        ISSN: 0883-0738            Impact factor:   1.987


  22 in total

Review 1.  Assessment in multisite randomized clinical trials of patients with autistic disorder: the Autism RUPP Network. Research Units on Pediatric Psychopharmacology.

Authors:  L E Arnold; M G Aman; A Martin; A Collier-Crespin; B Vitiello; E Tierney; R Asarnow; F Bell-Bradshaw; B J Freeman; P Gates-Ulanet; A Klin; J T McCracken; C J McDougle; J J McGough; D J Posey; L Scahill; N B Swiezy; L Ritz; F Volkmar
Journal:  J Autism Dev Disord       Date:  2000-04

2.  Autonomic nervous system dysregulation: breathing and heart rate perturbation during wakefulness in young girls with Rett syndrome.

Authors:  Debra E Weese-Mayer; Steven P Lieske; Christina M Boothby; Anna S Kenny; Heather L Bennett; Jean M Silvestri; Jan-Marino Ramirez
Journal:  Pediatr Res       Date:  2006-08-28       Impact factor: 3.756

3.  Rett syndrome: analysis of deaths in the British survey.

Authors:  A M Kerr; D D Armstrong; R J Prescott; D Doyle; D L Kearney
Journal:  Eur Child Adolesc Psychiatry       Date:  1997       Impact factor: 4.785

4.  Insulin-like growth factor 1 (IGF1) and its active peptide (1-3)IGF1 enhance the expression of synaptic markers in neuronal circuits through different cellular mechanisms.

Authors:  Aiden P Corvin; Ines Molinos; Graham Little; Gary Donohoe; Michael Gill; Derek W Morris; Daniela Tropea
Journal:  Neurosci Lett       Date:  2012-05-17       Impact factor: 3.046

5.  Neuroprotective effects of the N-terminal tripeptide of insulin-like growth factor-1, glycine-proline-glutamate (GPE) following intravenous infusion in hypoxic-ischemic adult rats.

Authors:  J Guan; G B Thomas; H Lin; S Mathai; D C Bachelor; S George; P D Gluckman
Journal:  Neuropharmacology       Date:  2004-11       Impact factor: 5.250

Review 6.  Neurophysiology of Rett syndrome.

Authors:  Daniel G Glaze
Journal:  J Child Neurol       Date:  2005-09       Impact factor: 1.987

7.  Effects of acetyl-L-carnitine on cardiac dysautonomia in Rett syndrome: prevention of sudden death?

Authors:  F Guideri; M Acampa; Y Hayek; M Zappella
Journal:  Pediatr Cardiol       Date:  2005 Sep-Oct       Impact factor: 1.655

8.  Rett syndrome in Australia: a review of the epidemiology.

Authors:  Crystal L Laurvick; Nicholas de Klerk; Carol Bower; John Christodoulou; David Ravine; Carolyn Ellaway; Sarah Williamson; Helen Leonard
Journal:  J Pediatr       Date:  2006-03       Impact factor: 4.406

9.  Specific mutations in methyl-CpG-binding protein 2 confer different severity in Rett syndrome.

Authors:  J L Neul; P Fang; J Barrish; J Lane; E B Caeg; E O Smith; H Zoghbi; A Percy; D G Glaze
Journal:  Neurology       Date:  2008-03-12       Impact factor: 9.910

10.  A randomized double-blind, placebo-controlled trial of minocycline in children and adolescents with fragile x syndrome.

Authors:  Mary Jacena S Leigh; Danh V Nguyen; Yi Mu; Tri I Winarni; Andrea Schneider; Tasleem Chechi; Jonathan Polussa; Paul Doucet; Flora Tassone; Susan M Rivera; David Hessl; Randi J Hagerman
Journal:  J Dev Behav Pediatr       Date:  2013-04       Impact factor: 2.225

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  21 in total

1.  A Psychometric Evaluation of the Motor-Behavioral Assessment Scale for Use as an Outcome Measure in Rett Syndrome Clinical Trials.

Authors:  Melissa Raspa; Carla M Bann; Angela Gwaltney; Timothy A Benke; Cary Fu; Daniel G Glaze; Richard Haas; Peter Heydemann; Mary Jones; Walter E Kaufmann; David Lieberman; Eric Marsh; Sarika Peters; Robin Ryther; Shannon Standridge; Steven A Skinner; Alan K Percy; Jeffrey L Neul
Journal:  Am J Intellect Dev Disabil       Date:  2020-11-01

Review 2.  Severity Assessment in CDKL5 Deficiency Disorder.

Authors:  Scott Demarest; Elia M Pestana-Knight; Heather E Olson; Jenny Downs; Eric D Marsh; Walter E Kaufmann; Carol-Anne Partridge; Helen Leonard; Femida Gwadry-Sridhar; Katheryn Elibri Frame; J Helen Cross; Richard F M Chin; Sumit Parikh; Axel Panzer; Judith Weisenberg; Karen Utley; Amanda Jaksha; Sam Amin; Omar Khwaja; Orrin Devinsky; Jeffery L Neul; Alan K Percy; Tim A Benke
Journal:  Pediatr Neurol       Date:  2019-03-27       Impact factor: 3.372

Review 3.  Clinical and biological progress over 50 years in Rett syndrome.

Authors:  Helen Leonard; Stuart Cobb; Jenny Downs
Journal:  Nat Rev Neurol       Date:  2016-12-09       Impact factor: 42.937

4.  Content Validation of Clinician-Reported Items for a Severity Measure for CDKL5 Deficiency Disorder.

Authors:  Jacinta Saldaris; Judith Weisenberg; Elia Pestana-Knight; Eric D Marsh; Bernhard Suter; Rajsekar Rajaraman; Gena Heidary; Heather E Olson; Orrin Devinsky; Dana Price; Peter Jacoby; Helen Leonard; Tim A Benke; Scott Demarest; Jenny Downs
Journal:  J Child Neurol       Date:  2021-08-11       Impact factor: 1.987

Review 5.  Clinician- and Patient-reported Endpoints in CNS Orphan Drug Clinical Trials: ISCTM Position Paper on Best Practices for Endpoint Selection, Validation, Training, and Standardization.

Authors:  Joan Busner; Gahan Pandina; SilviaZaragoza Domingo; Anna-Karin Berger; Maria T Acosta; Nahome Fisseha; Joseph Horrigan; Jelena Ivkovic; William Jacobson; Dennis Revicki; Victoria Villalta-Gil
Journal:  Innov Clin Neurosci       Date:  2021 Oct-Dec

Review 6.  Clinical Trial Design for Disease-Modifying Therapies for Genetic Epilepsies.

Authors:  Dylan C Brock; Scott Demarest; Tim A Benke
Journal:  Neurotherapeutics       Date:  2021-09-30       Impact factor: 6.088

7.  Pharmacological read-through of R294X Mecp2 in a novel mouse model of Rett syndrome.

Authors:  Jonathan K Merritt; Bridget E Collins; Kirsty R Erickson; Hongwei Dong; Jeffrey L Neul
Journal:  Hum Mol Genet       Date:  2020-08-29       Impact factor: 6.150

8.  Comparison of Core Features in Four Developmental Encephalopathies in the Rett Natural History Study.

Authors:  Clare Cutri-French; Dallas Armstrong; Joni Saby; Casey Gorman; Jane Lane; Cary Fu; Sarika U Peters; Alan Percy; Jeffrey L Neul; Eric D Marsh
Journal:  Ann Neurol       Date:  2020-06-29       Impact factor: 10.422

9.  Progress in Rett Syndrome: from discovery to clinical trials.

Authors:  Alan K Percy
Journal:  Wien Med Wochenschr       Date:  2016-08-04

10.  Development of the Tailored Rett Intervention and Assessment Longitudinal (TRIAL) database and the Rett Evaluation of Symptoms and Treatments (REST) Questionnaire.

Authors:  Paramala Santosh; Kate Lievesley; Federico Fiori; Jatinder Singh
Journal:  BMJ Open       Date:  2017-06-21       Impact factor: 2.692

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