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AI + NLP, Neurology, Rare Disease, RWE + HEOR

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.