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Lennox-Gastaut syndrome: a comprehensive review

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Abstract

Lennox-Gastaut syndrome (LGS) is considered an epileptic encephalopathy and is defined by a triad of multiple drug-resistant seizure types, a specific EEG pattern showing bursts of slow spike-wave complexes or generalized paroxysmal fast activity, and intellectual disability. The prevalence of LGS is estimated between 1 and 2% of all patients with epilepsy. The etiology of LGS is often divided into two groups: identifiable (genetic-structural-metabolic) in 65 to 75% of the patients and LGS of unknown cause in others. Lennox-Gastaut syndrome may be considered as secondary network epilepsy. The seizures in LGS are usually drug-resistant, and complete seizure control with resolution of intellectual and psychosocial dysfunction is often not achievable. Reduction in frequency of the most incapacitating seizures (e.g., drop attacks and tonic-clonic seizures) should be the major objective. Valproate, lamotrigine, and topiramate are considered to be the first-line drugs by many experts. Other effective antiepileptic drugs include levetiracetam, clobazam, rufinamide, and zonisamide. The ketogenic diet is an effective and well-tolerated treatment option. For patients with drug resistance, a further therapeutic option is surgical intervention. Corpus callosotomy is a palliative surgical procedure that aims at controlling the most injurious seizures. Finally, vagus nerve stimulation offers reasonable seizure improvement. The long-term outcome for patients with LGS is generally poor. This syndrome is often associated with long-term adverse effects on intellectual development, social functioning, and independent living.

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Authors and Affiliations

  1. Neurosciences Research Center, Shiraz Medical School, Shiraz University of Medical Sciences, Shiraz, Iran

    Ali A. Asadi-Pooya

  2. Jefferson Comprehensive Epilepsy Center, Department of Neurology, Thomas Jefferson University, Philadelphia, PA, USA

    Ali A. Asadi-Pooya

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Correspondence to Ali A. Asadi-Pooya.

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The author has the following disclosures: consultant: Cerebral Therapeutics, LLC and UCB Pharma; honorarium: Hospital Physician Board Review Manual and Cobel Daru; and royalty: Oxford University Press (book publication).

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Asadi-Pooya, A.A. Lennox-Gastaut syndrome: a comprehensive review. Neurol Sci 39, 403–414 (2018). https://doi.org/10.1007/s10072-017-3188-y

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