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Understanding Lennox–Gastaut syndrome: insights from focal epilepsy patients with Lennox–Gastaut features

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Abstract

To delineate the clinical and EEG features of adults with focal epilepsy associated with a generalized paroxysmal fast activity (GPFA) pattern on EEG who developed refractory seizures, notably drop attacks, but do not fulfill the classical triad for the diagnosis of Lennox–Gastaut syndrome (LGS) and provide further insight into LGS mechanisms. Among 957 patients admitted to video-EEG monitoring between 2002 and 2015, we retrospectively research adult patients with refractory focal epilepsy, drop attacks and GPFA on EEG. We collected demographic, anamnestic, and clinical data from medical records. We reviewed for all patients the interictal and ictal video-EEG recordings. We identified ten patients with focal epilepsy and electro-clinical features of LGS. As compared to classical LGS patients, our patients: (1) began epilepsy later (15.4 ± 8 years); (2) exhibited exclusively focal onset seizures, including drop attacks seizures linked to focal asymmetrical tonic posturing seizures; (3) had a stable cognition over time and (4) evolved favourably with a good secondary response to treatments in 80% of cases. Interestingly, all patients exhibited apparent diffuse interictal and ictal EEG abnormalities but a detailed analysis revealed that 50% had asymmetrical GPFA and 70% secondary bilateral synchrony processes. We may hypothesize here that a process of “secondary LGS” occurred which produced a worsening of seizures with the apparition of drop attacks and GPFA on EEG. This study brings arguments to consider that some cases of LGS could be linked to the development of a “secondary epileptic network” driven by a primary focal epileptic zone.

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Authors and Affiliations

  1. Epileptology Unit, AP-HP, GH Pitie-Salpêtrière-Charles Foix, 75013, Paris, France

    Sophie Dupont, Raluca Banica-Wolters, Vincent Navarro & Vi-Huong Nguyen-Michel

  2. Rehabilitation Unit, AP-HP, GH Pitie-Salpêtrière-Charles Foix, Hôpital de la Pitié-Salpêtrière, 75013, Paris, France

    Sophie Dupont

  3. Reference Center of Rare Epilepsies, Paris, France

    Isabelle An-Gourfinkel

  4. Brain and Spine Institute (ICM; INSERM UMRS1127, CNRS UMR7225, UPMC), 75013, Paris, France

    Sophie Dupont, Virginie Lambrecq, Vincent Navarro & Claude Adam

  5. Department of Clinical Neurophysiology, AP-HP, GH Pitie-Salpêtrière-Charles Foix, 75013, Paris, France

    Vincent Navarro & Vi-Huong Nguyen-Michel

  6. Sorbonne University, UPMC Univ. Paris 06, 75005, Paris, France

    Sophie Dupont & Vincent Navarro

  7. Hôpital de la Salpêtrière, 47, boulevard de l’Hôpital, 75651, Paris Cedex 13, France

    Sophie Dupont

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  1. Sophie Dupont
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  2. Raluca Banica-Wolters
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  3. Isabelle An-Gourfinkel
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  4. Virginie Lambrecq
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  7. Vi-Huong Nguyen-Michel
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Correspondence to Sophie Dupont.

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The study has been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki.

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Dupont, S., Banica-Wolters, R., An-Gourfinkel, I. et al. Understanding Lennox–Gastaut syndrome: insights from focal epilepsy patients with Lennox–Gastaut features. J Neurol 264, 1388–1396 (2017). https://doi.org/10.1007/s00415-017-8535-7

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  • DOI: https://doi.org/10.1007/s00415-017-8535-7

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