Lennox-Gastaut syndrome

Introduction
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By Mary C Spiciarich MD and Solomon L Moshe MD

Lennox-Gastaut syndrome is also known as or subsumes Akinetic epilepsy, Cryptogenic Lennox-Gastaut syndrome, Symptomatic Lennox-Gastaut syndrome. -ed.

Lennox-Gastaut syndrome is diagnosed in individuals with intractable epilepsy characterized by multiple seizure types, slow spike-wave pattern on EEG, and cognitive deterioration typically after first seizure onset. Seizure types include tonic seizures that mainly occur in sleep, atonic seizures, atypical absences, and myoclonic seizures. The condition may follow West syndrome, but has also been associated with various genetic and neurocutaneous syndromes, metabolic diseases, early infectious or ischemic insults, or may have an unknown etiology. Numerous interventions as well as medications have been studied in treating this condition, most recently clobazam, which may be effective in controlling drop attacks.

  • Lennox-Gastaut syndrome is defined by severe seizures of multiple types in infancy and childhood, cognitive impairment, on EEG by slow spike wave, and bursts of generalized fast activity in sleep.
  • Many associated conditions are identified as preceding West syndrome, such as various genetic syndromes and early CNS insults of various etiologies; however, many patients have unknown etiology.
  • Treatment options include medications, ketogenic diet, surgery, and devices such as vagal nerve stimulation; however, seizures remain difficult to control even when all of these modalities are employed.
  • Further study is needed to identify etiology in an effort to improve therapeutic targets and outcomes.

In This Article

Introduction
Historical note and nomenclature
Clinical manifestations
Clinical vignette
Etiology
Pathogenesis and pathophysiology
Epidemiology
Prevention
Differential diagnosis
Diagnostic workup
Prognosis
Management
Pregnancy
References cited
Contributors