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Background <p>Patients with focal epilepsy may over time experience a significantly unfavorable progression, developing an electroclinical profile that meets some or all criteria for Lennox–Gastaut syndrome (LGS). This study aimed to describe the electroclinical characteristics of these patients, whom we referred to as patients with LGS-like, and to determine their common and distinguishing features compared to classical LGS.</p> Methods <p>We retrospectively examined 2,130 adult-patients who underwent video-EEG monitoring from 2002 to 2024. LGS-like was defined as focal epilepsy with over the disease’s course: (1) a clinically unfavorable progression, (2) the development of generalized paroxysmal fast activity (GPFA) on EEG, and (3) another LGS criteria: tonic/atonic seizures, and/or cognitive and/or behavioral regression, and/or diffuse slow spike-and-wave (SSW) activity. Patients with LGS-like were classified into full or partial spectrum based on whether or not they met all the criteria for LGS.</p> Results <p>Thirty-four patients (1.6%) met the criteria for LGS-like syndrome (median age at seizure onset: 10.5 ± 6.9&#xa0;years [range 1–33]; median epilepsy duration: 15.5 ± 11&#xa0;years [range 1–45]). Among them, 21 patients had tonic seizures, three had atonic seizures, and one exhibited both. Eight patients showed cognitive and/or behavioral regression, while 29 patients displayed diffuse SSW patterns. Seven patients (20.5%) fulfilled the criteria for full-spectrum LGS. Compared with classical LGS, patients with LGS-like showed distinct features: (1) later seizure onset, (2) lower rate of cognitive regression (23.5%), (3) predominantly asymmetric tonic seizures and focal seizures during GPFA (62%), (4) frequent asymmetric GPFA (50%) and GPFA during wakefulness (53%), (5) high rate of SSW at adulthood (85%), though mostly asymptomatic, (6) normal EEG background activity (91%), and (7) favorable outcomes in 69% of cases. The only distinguishing factor between full-spectrum and partial-spectrum LGS was disease severity at the time of video-EEG: all full-spectrum LGS patients (100%) experienced daily seizures, compared with 55.6% of partial-spectrum patients (<i>p</i> = 0.0036). The similar favorable outcome in both groups (71% vs. 68%, <i>p</i> = 0.7) highlight the potential reversibility of the processes underlying LGS-like, regardless of the number of LGS diagnostic criteria met.</p> Conclusions <p>The evolution from focal epilepsy to a syndrome that met some or all criteria for LGS supports the existence of a pathological continuum between the underlying epileptic networks. Early identification of such patients may enable timely therapeutic adaptation and help prevent unfavorable outcomes.</p>

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Refractory focal epilepsy evolving into a Lennox–Gastaut syndrome-like

  • 期刊论文

  • 发表日期：

    2025年11月18日

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  • Thi Hong Phan Nguyen,
  • Vi-Huong Nguyen-Michel,
  • Isabelle Gourfinkel-An,
  • Marion Houot,
  • Valerio Frazzini,
  • Vincent Navarro,
  • Sophie Dupont

摘要

Background

Patients with focal epilepsy may over time experience a significantly unfavorable progression, developing an electroclinical profile that meets some or all criteria for Lennox–Gastaut syndrome (LGS). This study aimed to describe the electroclinical characteristics of these patients, whom we referred to as patients with LGS-like, and to determine their common and distinguishing features compared to classical LGS.

Methods

We retrospectively examined 2,130 adult-patients who underwent video-EEG monitoring from 2002 to 2024. LGS-like was defined as focal epilepsy with over the disease’s course: (1) a clinically unfavorable progression, (2) the development of generalized paroxysmal fast activity (GPFA) on EEG, and (3) another LGS criteria: tonic/atonic seizures, and/or cognitive and/or behavioral regression, and/or diffuse slow spike-and-wave (SSW) activity. Patients with LGS-like were classified into full or partial spectrum based on whether or not they met all the criteria for LGS.

Results

Thirty-four patients (1.6%) met the criteria for LGS-like syndrome (median age at seizure onset: 10.5 ± 6.9 years [range 1–33]; median epilepsy duration: 15.5 ± 11 years [range 1–45]). Among them, 21 patients had tonic seizures, three had atonic seizures, and one exhibited both. Eight patients showed cognitive and/or behavioral regression, while 29 patients displayed diffuse SSW patterns. Seven patients (20.5%) fulfilled the criteria for full-spectrum LGS. Compared with classical LGS, patients with LGS-like showed distinct features: (1) later seizure onset, (2) lower rate of cognitive regression (23.5%), (3) predominantly asymmetric tonic seizures and focal seizures during GPFA (62%), (4) frequent asymmetric GPFA (50%) and GPFA during wakefulness (53%), (5) high rate of SSW at adulthood (85%), though mostly asymptomatic, (6) normal EEG background activity (91%), and (7) favorable outcomes in 69% of cases. The only distinguishing factor between full-spectrum and partial-spectrum LGS was disease severity at the time of video-EEG: all full-spectrum LGS patients (100%) experienced daily seizures, compared with 55.6% of partial-spectrum patients (p = 0.0036). The similar favorable outcome in both groups (71% vs. 68%, p = 0.7) highlight the potential reversibility of the processes underlying LGS-like, regardless of the number of LGS diagnostic criteria met.

Conclusions

The evolution from focal epilepsy to a syndrome that met some or all criteria for LGS supports the existence of a pathological continuum between the underlying epileptic networks. Early identification of such patients may enable timely therapeutic adaptation and help prevent unfavorable outcomes.