What is the pathophysiology of Lennox-Gastaut syndrome (LGS)?

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

The pathophysiology of LGS is not known. No animal models exist.

A variety of possible pathophysiologies have been proposed. One hypothesis states that excessive permeability in the excitatory interhemispheric pathways in the frontal areas is present when the anterior parts of the brain mature.

Involvement of immunogenetic mechanisms in triggering or maintaining some cases of LGS is hypothesized. Although one study found a strong association between LGS and the human lymphocyte antigen class I antigen B7, a second study did not. No clear-cut or homogeneous metabolic pattern was noted in 2 separate reports of positron emission tomography (PET) studies in children with LGS.


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