Literature DB >> 32610249

Diagnostic gap in genetic epilepsies: A matter of age.

Angel Aledo-Serrano1, Irene García-Morales2, Rafael Toledano3, Adolfo Jiménez-Huete4, Beatriz Parejo5, Carla Anciones4, Ana Mingorance6, Primitivo Ramos7, Antonio Gil-Nagel4.   

Abstract

OBJECTIVE: This study aimed to evaluate the access to advanced diagnostic tests in patients with epilepsy and intellectual disability, with special focus on genetics.
METHODS: Patients with epilepsy and intellectual disability evaluated between 2016 and 2018 at the Epilepsy Unit of two hospitals in Madrid, Spain were included. The main inclusion criterion was an undetermined etiological diagnosis after clinical assessment, neuroimaging, and electroencephalogram (EEG).
RESULTS: Two hundred and five patients with epilepsy and intellectual disability were evaluated, with 124 fulfilling the inclusion criteria (mean age: 33.9 years). Regarding the etiological workup, advanced neuroimaging, prolonged video-EEG, and any type of genetic test had been performed in 58%, 41%, and 40%, respectively. An etiological diagnosis was reached in 18.5%. The workup was considered incomplete in 67%. Variables that showed the strongest association with an incomplete diagnostic workup in the multivariate analysis were current age and seizure freedom.
CONCLUSIONS: Despite the multiple implications of modern diagnostic techniques, especially genetic testing, there is a large proportion of patients with epilepsy and intellectual disability who do not have access to them. Older age and seizure freedom seem to be associated with the highest diagnostic gap.
Copyright © 2020 Elsevier Inc. All rights reserved.

Entities:  

Keywords:  Developmental and epileptic encephalopathies; Diagnostic barriers; Epilepsy genetic panel; Genetic testing; Intellectual disability; Precision medicine

Year:  2020        PMID: 32610249     DOI: 10.1016/j.yebeh.2020.107266

Source DB:  PubMed          Journal:  Epilepsy Behav        ISSN: 1525-5050            Impact factor:   2.937


  5 in total

Review 1.  Epilepsy Genetics and Precision Medicine in Adults: A New Landscape for Developmental and Epileptic Encephalopathies.

Authors:  Álvaro Beltrán-Corbellini; Ángel Aledo-Serrano; Rikke S Møller; Eduardo Pérez-Palma; Irene García-Morales; Rafael Toledano; Antonio Gil-Nagel
Journal:  Front Neurol       Date:  2022-02-17       Impact factor: 4.003

2.  The Charlotte Project: Recommendations for patient-reported outcomes and clinical parameters in Dravet syndrome through a qualitative and Delphi consensus study.

Authors:  Ángel Aledo-Serrano; Ana Mingorance; Vicente Villanueva; Juan José García-Peñas; Antonio Gil-Nagel; Susana Boronat; JoséÁngel Aibar; Silvia Cámara; María José Yániz; Luis Miguel Aras; Bárbara Blanco; Rocío Sánchez-Carpintero
Journal:  Front Neurol       Date:  2022-09-01       Impact factor: 4.086

3.  Effect of fenfluramine on seizures and comorbidities in SCN8A-developmental and epileptic encephalopathy: A case series.

Authors:  Ángel Aledo-Serrano; Borja Cabal-Paz; Elena Gardella; Pablo Gómez-Porro; Otilia Martínez-Múgica; Alvaro Beltrán-Corbellini; Rafael Toledano; Irene García-Morales; Antonio Gil-Nagel
Journal:  Epilepsia Open       Date:  2022-07-20

4.  Phenotypic homogeneity in childhood epilepsies evolves in gene-specific patterns across 3251 patient-years of clinical data.

Authors:  David Lewis-Smith; Shiva Ganesan; Peter D Galer; Katherine L Helbig; Sarah E McKeown; Margaret O'Brien; Pouya Khankhanian; Michael C Kaufman; Alexander K Gonzalez; Alex S Felmeister; Roland Krause; Colin A Ellis; Ingo Helbig
Journal:  Eur J Hum Genet       Date:  2021-05-24       Impact factor: 4.246

5.  The Genetic Diagnosis of Ultrarare DEEs: An Ongoing Challenge.

Authors:  Luciana Musante; Paola Costa; Caterina Zanus; Flavio Faletra; Flora M Murru; Anna M Bianco; Martina La Bianca; Giulia Ragusa; Emmanouil Athanasakis; Adamo P d'Adamo; Marco Carrozzi; Paolo Gasparini
Journal:  Genes (Basel)       Date:  2022-03-12       Impact factor: 4.096

  5 in total

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