Introduction/Purpose: Lennox-Gastaut Syndrome (LGS) is one of the most common refractory epilepsies of childhood with significant morbidity and mortality. However there is paucity of data of this syndrome in resource limited settings (RLS). We sought to delineate the phenomenology, diagnosis, aetiologies, management and outcomes of children referred with LGS in the Western Cape Province of South Africa. To further identify early clinical markers differentiating LGS from other types of epilepsy. Methods. This retrospective observational cohort study included all children between 1 to < 18 years of age in the neurology database with a referral label of LGS between 2000-2018. The group were critiqued for those who met the diagnostic criteria of LGS. Then were categorized into those with confirmed LGS and remainder were not- LGS. Data of the social demographics, age of seizure onset, etiology, preceding epileptic spasms, and semiology of epilepsy types, management interventions were reviewed to identify key diagnostic indicators to permit early and targeted interventions for children with this epilepsy syndrome. Results; Of 2551 children managed with epilepsy, 110 were suspected at presentation to have LGS of these 66 records were available for assessment. The median (IQR) age in months at presentation was 35(16.0-54.5) with a slight male (37/66) predominance. 43(65%) met the criteria LGS and 23(35%) were not-LGS. 34(52%) had no identifiable cause for their epilepsy whilst a structural and metabolic cause were identified in 25(38%) and 3(5%) respectively. Moderate or severe cognitive impairment was associated with LGS (OR 2.59, p = 0.02 and OR =3.15, p = 0.01) and so were tonic seizures (OR=4.03, p=0.04). The most common diagnoses in the not-LGS group were other types of DEE not meeting the LGS criteria (15%) and uncategorised epileptic syndromes 11%. Conclusion; Over third of the children in this cohort were erroneously referred with LGS early in their course. This has implications for their management and prognostic counselling. Identification of indicators such as tonic seizures and moderate or severe intellectual/cognitive impairment are useful early markers which support a diagnosis of LGS and could be viable for use in our setting.
| Identifer | oai:union.ndltd.org:netd.ac.za/oai:union.ndltd.org:uct/oai:localhost:11427/36064 |
| Date | 14 March 2022 |
| Creators | Sebunya, Robert |
| Contributors | Wilmhurst, Jo M |
| Publisher | Faculty of Health Sciences, Department of Paediatrics and Child Health |
| Source Sets | South African National ETD Portal |
| Language | English |
| Detected Language | English |
| Type | Master Thesis, Masters, MPhil |
| Format | application/pdf |
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