Lennox-Gastaut syndrome

Lennox-Gastaut syndrome  is characterised by brief tonic and atonic seizures, atypical absences, and a generalised interictal EEG pattern of spike and slow wave discharges. It accounts for 2,9% of all childhood epilepsy. Incidence peaks between 3 and 5 years of age. Cognitive and psychiatric impairment are frequent. About 30% of cases occur in previously healthy children; most result from neuronal migration disorders and hypoxic brain damage. About 40% of children have previous infantile spasms. Tonic seizures are particularly frequent during sleep.  Epilepsy patients who have tonic and atonic seizures when they are awake can violently collapse.  Atypical absences might translate in non-convulsive status, which can worsen cognitive deterioration. About 80% of patients continue to have seizures later in life, with symptomatic origin and early onset having the poorest outcome. Long-term follow-up report mortality rates of up to 17%. The optimum treatment for Lennox-Gastaut syndrome remains uncertain.

If you have a child who you think may have this condition and you would like us to review the diagnosis or provide genetic testing please contact us at neuroscience@meyer.it.