John Libbey Eurotext

Diagnosing Lennox-Gastaut syndrome in an adult and its direct impact in epilepsy care Volume 24, issue 4, August 2022

Figures

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  • Figure 2.

We report a 34-year-old male with intellectual disability and refractory, childhood-onset, non-lesional epilepsy featuring absence, generalized tonicclonic, and tonic seizures leading to falls. Seizure onset occurred at the age of 11 years, and tonic seizures did not appear until he was an adult. Notably, tonic seizures were mostly during sleep. Physical examination did not show dermatologic or ophthalmologic abnormalities. Current antiseizure drugs include zonisamide and cenobamate, and he underwent vagus nerve stimulator implantation and corpus callosotomy as an adult. EEG showed slow (≤2.5 Hz) generalized spike-and-wave discharges, and generalized paroxysmal fast activity (figures 1, 2). Brain MRI did not show any epileptogenic lesion; genetic testing was not pursued. The triad of intellectual disability, multiple seizure types including tonic seizures, and slow generalized spike-and-wave discharges is consistent with Lennox-Gastaut syndrome (LGS). Diagnosing this syndrome has direct implications in care including several new medication options [1, 2]. Notably, although LGS can typically be differentiated from other epilepsy syndromes (such as Doose, Dravet, West syndromes and atypical benign partial epilepsy) based on electroclinical features, achieving an accurate diagnosis may be challenging [3].

Test yourself

  • The electroclinical triad characteristic of Lennox-Gastaut syndrome includes (please select all that apply):
    • Intellectual disability
    • Multiple seizure types
    • 3-4 Hz generalized spike-and-wave-discharges
    • ≤2.5 Hz generalized spike-and-wave discharges
  • Lennox-Gastaut syndrome should be differentiated from other epilepsy syndromes including (please select all that apply):
    • Dravet syndrome
    • Doose syndrome
    • West syndrome
    • Atypical benign partial epilepsy
Note: Reading the manuscript provides an answer to all questions. Correct answers may be accessed on the website, www.epilepticdisorders.com.

Supplementary material.

Summary slides accompanying the manuscript are available at www.epilepticdisorders.com.

Disclosures.

F. Nascimento is an Associate Editor of Epileptic Disorders. D. Weber is on the Speaker’s Bureau for SK Life Science. E. Thiele reports no disclosures relevant to the manuscript.

Licence This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License