Abstracts

Rationale: Lennox-Gastaut syndrome (LGS) is a rare, severe childhood-onset developmental and epileptic encephalopathy characterized by multiple drug-resistant seizures and impaired quality of life (QoL). This study aimed to describe healthcare resource utilisation and quality of life (QoL) among the Lennox-Gastaut Syndrome (LGS) population in Europe, stratified by different age groups.

Methods: Data were drawn from the Adelphi LGS Disease Specific Programme™, a cross-sectional survey conducted in France, Germany, Italy, Spain, and the UK from July 2022 to August 2023. Neurologists and paediatric neurologists reported details on demographics, seizure frequency and type, antiseizure medications (ASMs), rescue medications (RMs), healthcare resource utilisation (HCRU) and QoL for consecutive patients with a confirmed LGS diagnosis. Results are presented for total LGS population, with data split by age (< 6, 6-18 and >18 years) and by seizure types (generalized tonic-clonic (GTC), “all drop” (tonic/atonic/GTC/secondary GTC), “non-drop” seizures, experienced in the past month).

Results: A total of 93 neurologists and 21 paediatric neurologists provided data for 454 patients with LGS across five countries: France (83), Germany (78), Italy (131), Spain (118), and the UK (44). Mean [SD; Standard Deviation] age was 16.0 [10.8], 65% were male, and mean [SD] age at first seizure was 4.0 [2.5] years. In the past month, 20% patients experienced GTC seizures, 75% drop seizures and 65% non-drop seizures. Physicians reported QoL as good for 31%, neutral 21% and poor for 47% of patients. Day-time seizures, mental impairment and night-time seizures had the greatest impact on patients’ QoL (Table 1).

Patients had a mean [SD] of 2.3 [1.5] ASM treatment regimens since diagnosis. The most prescribed ASMs were valproate, clobazam, lamotrigine, rufinamide, levetiracetam and cannabidiol. RMs were prescribed to 70% of patients. The most prescribed RMs were diazepam, midazolam. Past-year HCRU records showed: 16% of patients had been hospitalised at least once, of whom: 52% were admitted to treat a seizure-related complication, 27% for status epilepticus, 92% were admitted via the emergency room (ER) and 19% admitted to the intensive care unit (ICU) (Table 2).


Conclusions: Despite receiving multiple treatment regimens, and 70% being prescribed RMs, patients with LGS continue to experience GTC and drop seizures into adulthood. Hospitalisations persist for 16%, most of whom are admitted via ER and one-fifth to the ICU. Almost half of patients’ QoL is rated as “poor” by their physicians. Seizures remain both the most common reason for hospitalisations and having the greatest impact on patients’ QoL. These data suggest a further need for new therapies to better control seizures to reduce HCRU and improve QoL.

Funding: The Disease Specific Programme™ (DSP) is an Adelphi Real World product and all ownership of the data is retained. UCB were one of multiple subscribers to the LGS DSP dataset and have funded this secondary analysis of the DSP.