Lennox-Gastaut Syndrome of Unknown Cause: Clinical Characteristics of Patients in the Epilepsy Phenome/Genome Project

Lennox-Gastaut is a devastating childhood onset epilepsy syndrome with an unknown cause in up to 25% of patients. In the early view access of the journal Epilepsia, Dr. Widdess-Walsh representing an international consortium of the EPGP investigators presents phenotypic or clinical characteristics of Lennox-Gastaut syndrome patients who were part of the study.

In the study, 135 patients with Lennox-Gastaut with no known cause were enrolled from 19 EPGP centers in the United States and abroad.

• Lennox-Gastaut study subjects in this cohort had a broad range of onset of epilepsy from 1 to 13 years and had male predominance.
• There was also an association with normal development prior to seizure onset in 59.2% of patients. Despite the diagnosis, almost half of the adult patients with Lennox-Gastaut syndrome completed secondary school.
• Parents were cognitively normal.
• All subjects had EEG recordings with generalized epileptiform abnormalities with spike wave frequency range of 1 to 5 Hz whereas 8.1% of subjects had EEG studies with a normal posterior dominant rhythm.
• Almost 12% of patients evolved from West syndrome.

The authors concluded that Lennox-Gastaut syndrome has distinctive characteristics including a broad range of onset, male predominance, and often normal development prior to the onset of seizures. Cognitive achievements, such as completion of secondary school, are possible in half of adult patients.