Experts urge routine checks for nonseizure symptoms in LGS

A team of experts in Italy has proposed a framework for the routine monitoring of nonseizure symptoms in people with Lennox-Gastaut syndrome (LGS) throughout their lives.

While the framework is a good place to start, the researchers noted a need for better assessment tools to adequately capture these symptoms and inform better care.

The narrative review, “Beyond seizures: A multidimensional approach to non-seizure issues in Lennox–Gastaut syndrome. Insights from Italian experts,” was published in Epilepsia Open. 

Framework looks beyond seizure counts

“This work offers a practical route to move LGS care beyond seizure counts toward participation, resilience, and quality of life for patients and families,” the researchers wrote.

The hallmark of LGS is seizures — children with the rare form of epilepsy typically experience multiple types of treatment-resistant seizures, often daily.

In addition, a wide range of other symptoms can occur, including cognitive impairments and developmental delays, behavioral and social challenges, sleep difficulties, and motor impairments.

While these symptoms have a major impact on quality of life, they often go overlooked, according to the authors. Most available tools for measuring them were developed for a broader pediatric population, and don’t specifically target the unique issues of LGS.

“This gap between the living burden of patients and the tools available to clinicians creates a significant lack in care and risks overlooking domains that most strongly influence patient and caregiver outcomes,” the team wrote.

To fill that gap, a panel of experts reviewed current approaches to nonseizure issues in LGS and proposed a new framework to help clinicians more systematically monitor and address them.

Nonseizure issues should be discussed regularly

The experts said that doctors and families should discuss nonseizure issues at every clinic visit. This should be supplemented by formal assessments every six to 12 months or yearly, depending on the symptom area and the patient’s needs.

To build their recommendations, the team identified the nonseizure issues most relevant to people with LGS across different age groups.

For example, in early childhood, language development, alertness, and sleep are major concerns. In school-aged children, attention, learning, behavioral regulation, and motor control take center stage.

By adolescence, psychiatric symptoms, behavioral regulation, alertness, executive functioning, and social relationships become key concerns. In adulthood, independence in daily activities, occupational participation, and psychosocial frailty also become important.

The experts identified available clinical tools that may be suitable for evaluating each domain at different ages, and how often they should be administered based on the symptom’s relevance and typical trajectory in that age group.

They noted, however, that the strategy should be individualized. The specific tests, and their frequency, should be tailored to factors such as developmental stage, specific health concerns, disability level, and family priorities.

Tools vary in depth and feasibility

Certain tools may be easier to use during routine monitoring, while others that are more in-depth can be selected for patients who need more careful evaluation.

“Framed in this way, reexamination frequency is not arbitrary but represents a strategic tool to anticipate clinical needs, optimize interventions, and prevent both patient decline and caregiver exhaustion,” the researchers wrote.

They also added that assessments of the burden on caregivers and siblings should be conducted regularly.

While this framework may help avoid overlooking nonseizure symptoms, the available tools are not ideal for LGS, according to the authors. In addition to lacking specificity for the disease, the tools are limited in their ability to detect small but meaningful changes, and most are not designed to look at adult-specific concerns.

The scientists emphasized that optimal assessment tools should also combine quantitative measures with subjective insights from patients, caregivers, and teachers.

“The way forward requires validated, LGS-specific instruments that integrate clinical, cognitive, emotional, and social domains while systematically incorporating caregiver perspectives,” the researchers wrote.

“Tools should be sensitive to subtle changes, adaptable across developmental stages, and feasible for routine use, enabling a more comprehensive evaluation of both clinical care and treatment effects beyond seizure control.”

Better tools may clarify treatment effects

While treatments for LGS have traditionally been evaluated mainly for seizure control, there is preliminary evidence that some, including Fintepla (fenfluramine) and Epidiolex (cannabidiol), may have benefits on alertness, behavior, sleep, and communication.

Better symptom assessment tools could also help doctors better understand whether and how these medications affect nonseizure symptoms, the team noted.

“Ultimately, the goal is not only to prevent decline but also to promote resilience and well-being … for patients and families living with LGS,” the team concluded.