New insights highlight unmet needs for people living with Lennox-Gastaut syndrome and significant burden of the disease

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Sep 09, 2024, 01:00 ET

New data presented at the 15^th European Epilepsy Congress highlight lengthy delays in Lennox-Gastaut syndrome (LGS) diagnosis in Europe and significant disease burden from both seizure and non-seizure impairments^[1]
LGS is a severe childhood-onset developmental and epileptic encephalopathy, a group of epilepsies associated with developmental impairment, which is characterized by several different seizure types^[2]
New treatment options are needed to improve the long-term prognosis of individuals with LGS^[3]

BRUSSELS, Sept. 9, 2024 /PRNewswire/ -- The need for quicker and more accurate Lennox-Gastaut syndrome (LGS) diagnoses was highlighted today in a European real-world study presented at the 15^th European Epilepsy Congress using data from the Adelphi LGS Disease Specific Programme™ (DSP). Data from 454 pediatric and adult LGS patients across Europe reported it takes on average 12.3 months to receive a correct LGS diagnosis, following the first seizure at 4 years old (mean average age), exposing the lengthy wait patients and their families face for a correct diagnosis in Europe.^[1]

Ongoing challenges and unmet needs in LGS

Despite patients receiving more than three (3.4) antiseizure medications on average each day, ongoing challenges in effectively treating LGS remain^[1]:

71% of patients (N=324/454) experienced at least 1 concomitant comorbidity, with the most frequently reported including psychomotor or cognitive impairment, attention deficit hyperactivity disorder (ADHD), sleep disorder or insomnia
19% and 28% of patients experienced severe or very severe physical and mental impairment, respectively, many of which persist with age
47% of patients were reported to have at least somewhat poor quality of life
Daytime seizures were reported to have the greatest impact on quality of life for 39% of patients

These findings underline the need for new treatments that can target both drug-resistant seizures and the non-seizure symptoms of LGS.

Dr Konrad Werhahn, Global Medical Affairs, UCB, said: "The impact of LGS goes beyond seizures and can include severe cognitive impairment, communication difficulties, psychiatric symptoms, behavioral challenges, and mobility problems, all of which constitute a major burden for patients and their caregivers/families. At UCB, we understand the need for a multidisciplinary, individualized approach to care which addresses each patient's medical, educational, psychological, and social needs throughout the course of their life."

LGS affects approximately one million people worldwide.^[4] However, diagnosis remains complex and challenging due to the lack of specific biological markers for the condition, multiple possible causes, and varied presentation of symptoms.^[2]

Although several approved drugs are available for LGS, there are still unmet needs for treatment. These drugs provide an improvement, but usually not with a sustained long-term effect, resulting in a need for novel therapies which effectively target seizures and alleviate non-seizure outcomes to enhance the prognosis for patients with LGS.^[3]

Diagnostic aid for healthcare professionals

In addition, at the 15th European Epilepsy Congress, details of a new LGS electronic decision-assisting tool based on International League Against Epilepsy (ILAE) diagnostic criteria were presented. Developed by a group of ten epilepsy experts, its digitalization has been funded by UCB (with no influence on its content), the tool is designed to help physicians evaluate the likelihood that their patient has LGS. The questionnaire considers the mandatory, alert, and exclusionary features of LGS, providing a result that will guide future management. The prototype will now be tested and validated prior to use by healthcare professionals in the clinical setting.

About UCB

UCB, Brussels, Belgium (www.ucb.com) is a global biopharmaceutical company focused on the discovery and development of innovative medicines and solutions to transform the lives of people living with severe diseases of the immune system or of the central nervous system. With approximately 9,000 people in approximately 40 countries, the company generated revenue of €5.3 billion in 2023. UCB is listed on Euronext Brussels (symbol: UCB). Follow us on Twitter: @UCB_news.

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References:

^[1]Strzelczyk A, Gil-Nagel A, Striano P et al. Insights into Lennox-Gastaut syndrome: A European real-world study on patient profiles and unmet needs. Poster presented at: The 15th European Epilepsy Congress (EEC); 2024, September 7-11; Rome, Italy.
^[2]Bourgeois BFD, Douglass LM, Sankar R. et al Lennox-Gastaut syndrome: A consensus approach to differential diagnosis. Epilepsia. 2014;55(Suppl 4):4-9.
^[3] Auvin S. Lennox-Gastaut syndrome: New treatments and treatments under investigation. Rev Neurol (Paris). 2020;176(6):444-7.
^[4] LGS Foundation. Factsheet: What is LGS? https://www.lgsfoundation.org/wp-content/uploads/2024/05/Updated-MAY-2024.png. Last accessed: July 2024.
^{^[5]} Arzimanoglou A, Specchio N, Auvin S et al. Development of an Electronic Diagnostic Criteria Tool for Lennox-Gastaut Syndrome (LGS). Poster presented at: The 15th European Epilepsy Congress (EEC); 2024, September 7-11; Rome, Italy.
^{^[6]} Specchio N, Wirrell EC, Scheffer IE, et al. ILAE Classification and Definition of Epilepsy Syndromes with Onset in Childhood: Position Paper by the ILAE Task Force on Nosology and Definitions. Epilepsia. 2022;63(6):1398-442.