Bermúdez ML, Moreno AÁJ

Language: Spanish
References: 10
Page: 56-66
PDF size: 475.11 Kb.

ABSTRACT

Lennox-Gastaut syndrome (LGS) is one of the most severe epileptic encephalopathies of childhood, characterized by an electroclinical triad of generalized spike and wave (SW) activity in the electroencephalogram (EEG), multiple types of seizures and mental regression. By its etiology, it is subdivided into symptomatic (secondary to an underlying brain disorder) and cryptogenic (unknown cause) types. Twenty percent of all patients with LGS have prior history of West syndrome. The characteristic interictal EEG pattern is 1.5 to 2.5 Hz SW bilaterally and synchronous activity, dominant over of frontocentral regions. There are varying degrees of slowing of the background. Paroxysms of generalized fast rhythmic activity (10 to 25 Hz) are frequently seen during sleep. The most frequent seizures include tonic seizures, atypical absence seizures and drop attacks, and frequent presentation of status epilepticus. There are few effective treatment options, since it is highly refractory to antiepileptic drugs (AEDs), therefore the prognosis of these patients is generally poor despite of new therapeutic approach, which includes in addition to AEDs, «off label» medications, immune therapy, ketogenic diet and surgery.

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