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The 2017 and 2022 ILAE epilepsy classification systems identify needs and opportunities in care: A paediatric
Eoin P Donnellan1, Caroline Kehoe1, Ailbhe Moran1
1Department of Paediatrics, Galway University Hospital, Ireland.
Insights
The 2017 and 2022 International League Against Epilepsy (ILAE) classification systems reveal significant neurodevelopmental comorbidities and genetic causes in childhood epilepsy. However, a substantial gap exists between identifying epilepsy causes and implementing effective precision therapies.
Area of Science:
- Pediatric Neurology
- Epileptology
- Clinical Genetics
Background:
- The International League Against Epilepsy (ILAE) updated classification systems in 2017 and 2022.
- There is limited data on applying these updated ILAE classifications in routine clinical practice for childhood epilepsy.
- Understanding epilepsy spectrum, etiology, comorbidities, and the role of molecular diagnostics is crucial for advancing care.
Purpose of the Study:
- To evaluate the application of the 2017 and 2022 ILAE epilepsy classification systems in a pediatric hospital cohort.
- To identify epilepsy spectrum, etiologies, comorbidities, and the utility of molecular genetic diagnosis.
- To assess the availability and impact of precision therapies based on molecular diagnoses.
Main Methods:
- A cross-sectional, retrospective study of children (≤16 years) with epilepsy from 2017-2022 at University Hospital Galway.
- Standardized data collection and analysis using the 2017 and 2022 ILAE classification criteria.
- Review of etiological factors, comorbidities, and molecular genetic diagnostic results.
Main Results:
- Epilepsy was classified as focal (46.1%), generalized (38.8%), or combined (6.2%).
- Epilepsy syndromes were identified in 40.7% of cases, with SeLECTS being most common.
- Molecular diagnosis was confirmed in 19.9%, with a presumed genetic etiology in an additional 35.7%. Significant comorbidities included global delay (29.2%) and ASD (14.6%). Precision therapies were available for 5.9% and utilized in 3.7%.
Conclusions:
- The latest ILAE classification systems facilitate comparison across settings and highlight high rates of neurodevelopmental comorbidities and genetic etiologies in childhood epilepsy.
- A significant gap exists between identifying the cause of epilepsy and implementing effective disease-modifying precision therapies.
- The 2017/2022 ILAE classifications effectively identify challenges in routine epilepsy care, emphasizing the need for improved therapeutic strategies.
Objectives:
There is a paucity of studies reporting the epilepsy spectrum using the 2017 and 2022 ILAE classification systems in everyday clinical practice. To identify gaps and opportunities in care we evaluated a hospital-based cohort applying these epilepsy classification systems, including aetiology and co-morbidity, and the utility of molecular genetic diagnosis to identify available precision therapies.
Methods:
Cross sectional retrospective study of all children with epilepsy (≤16 years) attending University Hospital Galway (2017-2022). Data collection and analysis of each case was standardised to ensure a systematic approach and application of the recent ILAE categorisation and terminology (2017 and 2022). Ethics approval was obtained.
Results:
Among 356 children, epilepsy was classified as focal (46.1 %), generalised (38.8 %), combined (6.2 %), and unknown (9 %). Epilepsy syndrome was determined in 145/356 (40.7 %), comprising 24 different syndromes, most commonly SeLECTS (9 %), CAE (7 %), JAE (6.2 %) and IESS (5.9 %). New aetiology-specific syndromes were identified (e.g. CDKL5-DEE). Molecular diagnosis was confirmed in 19.9 % (n = 71) which encompassed monogenic (13.8 %) and chromosomopathy/CNV (6.2 %). There was an additional 35.7 % (n = 127) of patients who had a presumed genetic aetiology of epilepsy. Remaining aetiology included structural (18.8 %, n = 67), infectious (2 %, n = 7), metabolic (1.7 %, n = 6) and unknown (30.3 %, n = 108). Encephalopathy categorisation was determined in 182 patients (DE in 38.8 %; DEE in a further 11.8 %) associated with a range of co-morbidities categorised as global delay (29.2 %, n = 104), severe neurological impairment (16.3 %, n = 58), and ASD (14.6 %, n = 52). Molecular-based "precision therapy" was deemed available in 21/356 (5.9 %) patients, with "molecular precision" approach utilised in 13/356 (3.7 %), and some benefit noted in 6/356 (1.7 %) of overall cohort or 6/71 (8.5 %) of the molecular cohort.
Conclusion:
Applying the latest ILAE epilepsy classification systems allow comparison across settings and identifies a major neuro-developmental co-morbidity rate and a large genetic aetiology. We identified very few meaningful molecular-based disease modifying "precision therapies". There is a monumental gap between aetiological identification, and impact of meaningful therapies, thus the new 2017/2022 classification clearly identifies the major challenges in the provision of routine epilepsy care.
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