Development and testing of methods to record and follow up spells in patients with alternating hemiplegia of childhood.

Autor: Sentmanat MK; Duke University Department of Pediatrics, Division of Pediatric Neurology and Developmental Medicine, Durham, NC, USA., Papadopoulou MT; Department of Pediatric Clinical Epileptology, Sleep Disorders and Functional Neurology, University Hospitals of Lyon (HCL), Lyon, France; EpiCARE-ERN Full Member, Italy., Prange L; Duke University Department of Pediatrics, Division of Pediatric Neurology and Developmental Medicine, Durham, NC, USA., Fons C; EpiCARE-ERN Full Member, Italy; Department of Child Neurology, Sant Joan de Déu Children's Hospital, Barcelona, Spain., De Grandis E; EpiCARE-ERN Full Member, Italy; Child Neuropsychiatry Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy; Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DiNOGMI), University of Genoa, Genoa, Italy., Vezyroglou A; Department of Developmental Neurosciences, UCL NIHR BRC Great Ormond Street Institute of Child Health, London, UK., Boggs A; Duke University Department of Pediatrics, Division of Pediatric Neurology and Developmental Medicine, Durham, NC, USA., Su S; Duke University Department of Pediatrics, Division of Pediatric Neurology and Developmental Medicine, Durham, NC, USA., Comajuan M; Department of Pediatric Clinical Epileptology, Sleep Disorders and Functional Neurology, University Hospitals of Lyon (HCL), Lyon, France; EpiCARE-ERN Full Member, Italy., Wuchich J; CureAHC Foundation, USA., Jóhannesson S; Iceland AHC Foundation, Italy., Huaynate JA; Department of Child Neurology, Sant Joan de Déu Children's Hospital, Barcelona, Spain., Stagnaro M; Child Neuropsychiatry Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy; Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DiNOGMI), University of Genoa, Genoa, Italy., Megvinov A; Euro Mediterranean Institute of Science and Technology I.E.ME.S.T., Palermo, Italy., Patel S; Duke University Department of Pediatrics, Division of Pediatric Neurology and Developmental Medicine, Durham, NC, USA., Arzimanoglou A; Department of Pediatric Clinical Epileptology, Sleep Disorders and Functional Neurology, University Hospitals of Lyon (HCL), Lyon, France; EpiCARE-ERN Full Member, Italy., Vavassori R; EpiCARE-ERN Full Member, Italy; Euro Mediterranean Institute of Science and Technology I.E.ME.S.T., Palermo, Italy; Association AHC18+ e.V., Germany., Panagiotakaki E; Department of Pediatric Clinical Epileptology, Sleep Disorders and Functional Neurology, University Hospitals of Lyon (HCL), Lyon, France; EpiCARE-ERN Full Member, Italy., Mikati MA; Duke University Department of Pediatrics, Division of Pediatric Neurology and Developmental Medicine, Durham, NC, USA; Department of Neurobiology, Duke University, Durham, NC, USA. Electronic address: mohamad.mikati@duke.edu.
Jazyk: angličtina
Zdroj: European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society [Eur J Paediatr Neurol] 2023 Sep; Vol. 46, pp. 98-107. Date of Electronic Publication: 2023 Aug 04.
DOI: 10.1016/j.ejpn.2023.07.005
Abstrakt: Background: Developing methods to record Alternating Hemiplegia of Childhood (AHC) spells is essential for clinical trials and patient care.
Objectives: Test the following hypotheses: 1) Video-library training improves participants' ability to correctly identify AHC spells. 2) A custom-designed event-calendar with weekly reviews results in consistent documentation of such events over time. 3) Use of an electronic diary (e-Diary) to register events is a useful tool.
Methods: 1) A video-library of AHC type spells was developed along with specific training; the effect of the training was tested in 36 caregivers. 2) An event-calendar was similarly developed and provided to 5 caregivers with weekly videoconference meetings for 8 weeks. 3) An e-Diary was developed and offered to 33 patients; time of usage and caregivers' feedback (telephone interview) were analyzed.
Results: 1) Video-library training: Wilcoxon test showed improvement in caregiver identification of spells (p = 0.047), Cohen's Kappa demonstrated high degree of agreement between caregivers'-experts' classifications (>0.9). 2) Event-calendar: 96.42% of entries had complete information; this did not change during follow up (p = 0.804). 3) e-Diary: whereas 52% of respondents used the e-Diary when offered (duration: 10.5 ± 8.1 months), 96.3% indicated they would use it in future studies. Those who used it for 13 months, were very likely to use it during the rest of that year.
Conclusions: Video-library training improved spell identification. Calendar with weekly reviews resulted in a sustained and consistent record keeping. Caregivers' e-Diary feedback was encouraging with long-term usage in many. These approaches could be helpful for AHC and, potentially, in similar disorders.
Competing Interests: Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests:One of the authors (MAM) has intellectual property interest in gene therapy of ATP1A3 related disease.
(© 2023 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.)
Databáze: MEDLINE
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