Format

Send to

Choose Destination
J Neurol. 2017 Jul;264(7):1388-1396. doi: 10.1007/s00415-017-8535-7. Epub 2017 Jun 5.

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

Author information

1
Epileptology Unit, AP-HP, GH Pitie-Salpêtrière-Charles Foix, 75013, Paris, France. sophie.dupont@aphp.fr.
2
Rehabilitation Unit, AP-HP, GH Pitie-Salpêtrière-Charles Foix, Hôpital de la Pitié-Salpêtrière, 75013, Paris, France. sophie.dupont@aphp.fr.
3
Brain and Spine Institute (ICM; INSERM UMRS1127, CNRS UMR7225, UPMC), 75013, Paris, France. sophie.dupont@aphp.fr.
4
Sorbonne University, UPMC Univ. Paris 06, 75005, Paris, France. sophie.dupont@aphp.fr.
5
Hôpital de la Salpêtrière, 47, boulevard de l'Hôpital, 75651, Paris Cedex 13, France. sophie.dupont@aphp.fr.
6
Epileptology Unit, AP-HP, GH Pitie-Salpêtrière-Charles Foix, 75013, Paris, France.
7
Reference Center of Rare Epilepsies, Paris, France.
8
Brain and Spine Institute (ICM; INSERM UMRS1127, CNRS UMR7225, UPMC), 75013, Paris, France.
9
Department of Clinical Neurophysiology, AP-HP, GH Pitie-Salpêtrière-Charles Foix, 75013, Paris, France.
10
Sorbonne University, UPMC Univ. Paris 06, 75005, Paris, France.

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.

KEYWORDS:

Drop attacks; Focal epilepsy; Generalized paroxysmal fast activity; Secondary Lennox–Gastaut

PMID:
28584915
DOI:
10.1007/s00415-017-8535-7
[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Springer
Loading ...
Support Center