Lennox-Gastaut syndrome (LGS) is a rare disease commonly diagnosed in patients younger than 8 years old. LGS is usually associated with symptoms such as seizures, intellectual disability, and abnormal electroencephalogram (EEG) patterns. Although it is typically diagnosed in childhood, there are cases of patients being diagnosed later in life however, this is difficult to identify due to the range of different symptoms adult patients can experience.
This feasibility study will aim to understand the characteristics and number of adults (>18 years old) in the LGS population in England. Specifically, their age at diagnosis of LGS, age at first epilepsy diagnosis, age at diagnosis of their first seizure, Age at diagnosis of another condition that has been linked to LGS, Age when patients where prescribed their first medication for LGS, Age at first abnormal EEG performed and sex.
The results of this study will help understand the true number and unmet need in LGS patients in England that may have been previously under reported due to difficulties in identification and can be used to explore expanding the use of LGS drug treatments for patients who are impacted by LGS-like symptoms.
This is an observational retrospective cohort study to quantify the number and estimate the prevalence of confirmed LGS cases and LGS-like patients in the UK. The study comprises a study period from 2017 to 2020 and includes all patients diagnosed with LGS or LGS-like conditions at any age during the patient-specific study ascertainment period (from data availability start to the first of: end of study period or end of data availability) and aged 18+ years during the study period. LGS-like patients will consist of patients with a diagnosis of epilepsy during the study ascertainment period, at least one record of a tonic seizure during the ascertainment period, and at least one of the following: prescription of at least one LGS medication within 12 months of epilepsy diagnosis, diagnosis of at least one LGS comorbidity during the ascertainment period, and a record of at least one abnormal EEG performed during the ascertainment period. Feasibility study objectives include identifying the number of adult LGS and LGS-like patients in CPRD-HES, estimating the prevalence of adult LGS and LGS-like patients in CPRD-HES, and describing patient demographics and clinical characteristics of LGS and LGS-like patients, separately.
The feasibility analyses will calculate the total number of LGS and LGS-like patients identified in CPRD-HES during the study period and per each calendar year and prevalence will be calculated using the number of LGS and LGS-like patients identified from CPRD-HES divided by the total number of patients within CPRD-HES in the same time period. Patient demographic and clinical characteristics will be described in LGS and LGS-like patients separately. Descriptive analyses will be conducted using number and percent (N, %) for categorical variables and using mean (SD), median (IQR), and other relevant summary statistics for continuous variables.
1. Recordings of tonic seizures; Recordings of Abnormal EEG performance; LGS Comorbidities (Developmental delay, sleep disorder, hyperactivity, aggression, Autism spectrum disorder); LGS Medications (Rufinamide, Felbamate, Clobazam, Epidyolex®); Age at epilepsy; Age at LGS diagnosis; Age at first tonic seizure; Age at first diagnosis of LGS comorbidity; Age at first LGS medication initiation; Age at first record of abnormal EEG performed; sex
Sophia Fleming - Chief Investigator - IQVIA Ltd ( UK )
Christelle Elia - Corresponding Applicant - IQVIA Ltd ( UK )
GIANLUCA LUCREZI - Collaborator - IQVIA Solutions Italy S.r.l
Jessica Lundbom - Collaborator - IQVIA Ltd ( UK )
Rachel Armstrong - Collaborator - IQVIA Ltd ( UK )
HES Admitted Patient Care;HES Outpatient