Clinical burden and treatment patterns associated with Dravet or Lennox-Gastaut syndromes: A retrospective study using natural language processing of narrative unstructured electronic health records

Introduction: This study aimed to investigate the clinical and humanistic burden in two developmental and epileptic encephalopathies, Dravet syndrome and Lennox-Gastaut syndrome, and describe challenges related to treatment with antiseizure medications. Methods: Data were included from medical transcription records of patients with confirmed diagnoses of Dravet syndrome or Lennox-Gastaut syndrome undergoing routine care in the US between January 2010 and January 2022. Natural language processing technology was used to extract de-identified data related to demographics, seizure and nonseizure burden, impacts on quality of life, and treatment patterns. Results: The analysis included 166 patients with Dravet syndrome and 1063 patients with Lennox-Gastaut syndrome who were treated with ≥1 antiseizure medication. Mean age at diagnosis was 1.1 years for Dravet syndrome and 2.1 years for Lennox-Gastaut syndrome. Seizure burden was reported for almost all patients in both cohorts, with the most common seizure type being convulsive/tonic-clonic (Dravet syndrome: 42 % [pediatric], 35 % [adult]; Lennox-Gastaut syndrome: 35 % [pediatric], 26 % [adult]). The most frequent comorbidities in patients with Dravet syndrome were autism spectrum disorder (22 % [pediatric]) and cardiovascular disease (50 % [adult]), and in patients with Lennox-Gastaut syndrome were brain abnormalities (23 % [pediatric]) and cardiovascular disease (32 % [adult]). The most frequent nonseizure symptom was delayed communication (Dravet syndrome: 31 % [pediatric], 40 % [adult]; Lennox-Gastaut syndrome: 42 % [pediatric], 37 % [adult]). Problems with sleep, feeding, and mobility were found to be predominant detriments to quality of life. The most common antiseizure medications used were valproic acid (Dravet syndrome: 57 %; Lennox-Gastaut syndrome: 40 %), clobazam (Dravet syndrome: 53 %; Lennox-Gastaut syndrome: 49 %), and levetiracetam (Dravet syndrome: 52 %; Lennox-Gastaut syndrome: 47 %). Treatment changes occurred in 39 % and 28 % of patients with Dravet syndrome and Lennox-Gastaut syndrome, respectively, and were frequently attributed to adverse events (Dravet syndrome: 57 %; Lennox-Gastaut syndrome: 53 %) and efficacy considerations (Dravet syndrome: 26 %; Lennox-Gastaut syndrome: 30 %). Discussion: As assessed by natural language processing, this study revealed that patients with Dravet syndrome or Lennox-Gastaut syndrome treated with currently available antiseizure medications experience substantial seizure and nonseizure burden. Changes in antiseizure medication are common, often due to adverse events and efficacy considerations, suggesting the need for more effective and well-tolerated treatments.