What is the Lennox-Gastaut syndrome (LGS)?

Updated: Nov 09, 2018
  • Author: Koshi A Cherian, MD; Chief Editor: Amy Kao, MD  more...
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Answer

Childhood epileptic encephalopathy, or Lennox-Gastaut syndrome (LGS), is a devastating pediatric epilepsy syndrome constituting 1-4% of childhood epilepsies. [1] The syndrome is characterized by multiple seizure types; mental retardation or regression; and abnormal findings on electroencephalogram (EEG), with paroxysms of fast activity and generalized slow spike-and-wave discharges (1.5-2 Hz).

The most common seizure types are tonic-axial, atonic, and absence seizures, but myoclonic, generalized tonic-clonic, and partial seizures can be observed (see Clinical Presentation). An EEG is an essential part of the workup for LGS. Neuroimaging is an important part of the search for an underlying etiology (see Workup).

A variety of therapeutic approaches are used in LGS, ranging from conventional antiepileptic agents to diet and surgery (see Treatment and Management). Unfortunately, much of the evidence supporting these approaches is not robust, and treatment is often ineffective.


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