Resumo

HERRERA, Manuel L  e  BURNEO, Jorge G. Lennox Gastaut Syndrome: Diagnosis approach and therapeutics advances: Antiepileptic drugs, cannabidiol and other alternatives. Rev Neuropsiquiatr [online]. 2018, vol.81, n.2, pp.82-94. ISSN 0034-8597.  http://dx.doi.org/https://doi.org/10.20453/rnp.v81i2.3337.

Lennox Gastaut syndrome is a catastrophic childhood-onset epileptic encephalopathy that presents a variety of electroclinical features: 1) Multiple seizure types, particularly tonic ones; 2) Cognitive impairment associated with behavioral disturbances; 3) Slow spike-wave complex on electroencephalographic (EEG) recordings, and generalized fast rhythms during sleep. The syndrome’s etiology can be structural or genetic. Diagnosis at the time of clinical onset may be a challenge as not all criteria are met and there may not be a full picture; the differential diagnosis should consider childhood-onset myoclonic epilepsies. Treatment is equally complex as there are no clinically practical guidelines, reason for which experts’ opinions must be sought. Initial treatment with valproic acid is suggested. Lamotrigine, felbamate, topiramate and clobazam are second line agents approved by the USA Food and Drug Administration (FDA). Surgical management may include resective surgery and/or corpus callosotomy (complete or partial). Other alternatives include Vagus Nerve Stimulation (VNS), ketogenic diet, Deep Brain Stimulation and Cannabis-based treatment.

Palavras-chave : Lennox-Gastaut; refractory epilepsy; tonic seizure; fast rhythms; Cannabidiol.

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