Exploring The Clinical Burden Of Dravet Syndrome & Lennox-Gastaut Syndrome

Analysis Of Physician Notes To Examine Clinical & Humanistic Burden Of Patients With Dravet Syndrome & Lennox-Gastaut Syndrome In The United States

This poster was originally presented at the American Epilepsy Society (AES) Annual Meeting, December 1-5, 2023, in Orlando, FL.   

Authors: Satish Rao, Dave Iwanyckyj, Sally W. Wade, Pablo Racana, Fernando Otalora, Mei Lu 

Affiliations: Takeda Pharmaceuticals USA, Inc., Lexington, MA, USA; Amplity, Langhorne, PA, USA; Wade Outcomes Research and Consulting, Salt Lake City, UT, US 

Introduction

• Dravet Syndrome (DS) and Lennox-Gastaut Syndrome (LGS) are rare, childhood-onset, developmental epileptic encephalopathies that are distinguished by unpredictable and frequent seizures. 

• Despite recent advancements of clinical and nonclinical interventions, patients with DS and LGS continue to experience substantial burden in their intellectual development, social functioning, and quality of life (QOL). 

• The objective of this study is to characterize the clinical and humanistic burden of DS and LGS, including seizure and nonseizure burden and patients’ QOL. 

Methods

• Used medical transcriptions from AI & NLP-powered Amplity AnswerY™ (formerly Amplity Insights™), capturing routine care in the United States (US) from January 2010 to January 2022. 

• The database of approximately 55 million medical transcription records included inpatient and outpatient electronic, unstructured, records from approximately 25 million patients and 150,000 multispecialty physicians.  

• Natural language processing (NLP) technology retrospectively analyzed physician–patient interactions recorded in the database.  

• Eligible patients had ≥ 1 inpatient or outpatient record and a diagnosis of DS or LGS, based on symptoms and/or genetic or electroencephalography findings, and mention of treatment with ≥ 1 s antiseizure medications (ASM). 

Results

• As shown in Table 1, NLP queries identified 166 patients with Dravet Syndrome and 1063 patients with Lennox-Gastaut Syndrome who were treated with ≥ 1 ASM. 
• Overall, among patients with recorded data at the time of the healthcare provider visit (DS: n = 121; LGS: n = 877). 

• Pediatric patients aged < 18 years were more prevalent in the DS group (83%) and adult patients aged ≥ 18 years were more prevalent in the LGS group (58%).  

• Mean age at diagnosis for patients with recorded data (DS: n = 9; LGS: n = 53) was 1.1 years for patients with DS and 2.1 years for those with LGS. 

According to Figure 1:

• Half (50%) of the patients with Dravet Syndrome mentioned comorbidities, with the 3 most commonly reported being autism spectrum disorder (19%), cardiovascular disease (CVD) (15%), and insomnia/sleeping disorders (12%).  

• Among patients with Lennox-Gastaut Syndrome, 63% reported comorbidities, with CVD (21%), cerebral palsy (19%), and autism spectrum disorder (16%) being the 3 most common. 

According to Figure 2:

• A total of 164 (99%) patients with DS and 1036 (97%) patients with LGS reported seizures.  

• Among the reported seizures in patients with DS or LGS, causes of onset were unknown in 48% and 55%; generalized in 38% and 38%; and focal in 15% and 7%, respectively. 

 According to Figure 3:

• QOL impacts on patients included sleep issues (DS: 13%; LGS: 10%), feeding-tube use (DS: 8%; LGS: 8%), and inability or difficulty walking or sitting and/or requiring mobility aids (DS: 3%; LGS: 4%). 

• Caregivers’ burden was mentioned in records for 8% of patients with Dravet Syndrome and 14% of patients with Lennox-Gastaut Syndrome. 

Conclusion

• AnswerY analysis revealed that despite treatment with ASMs, patients with Dravet Syndrome or Lennox-Gastaut Syndrome experience seizures, nonseizure symptoms, and QOL burdens that extend to caregivers. 

• Application of NLP to future research merits further consideration. 

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