Lennox-Gastaut syndrome (LGS) is one of the most severe types of childhood epilepsy. It is usually resistant to treatment and associated with mental retardation. To delineate the risk factors associated with the outcome of LGS, we evaluated, in a retrospective and multicentre study, the course of the disease, EEG tracings, and intellectual function in 101 patients. Inclusion criteria were the presence of tonic seizures as well as slow spike and wave complexes in the EEG. The average documented observation period was 16 years (range 4 - 31 years). Overall, the intellectual and neurological outcome was poor. At the last follow-up, 38 % of the patients could not speak, 21 % were unable to walk and only 4 % were free of seizures. Four independent risk factors for severe mental retardation were identified by multivariate analysis. These were in a decreasing order of importance: nonconvulsive status epilepticus (NCSE), odds ratio (OR) 25.2, a previous diagnosis of West syndrome (OR 11.6), a symptomatic etiology of epilepsy (OR 9.5), and an early age at onset of epilepsy (OR 4.7). The results highlight the association between NCSE and the severity of mental retardation in patients with LGS; this association appears to be independent of symptomatic etiology. Our data provide an indirect evidence that, at least in some of the patients, NCSE is not only a concomitant feature, but also a cause of severe mental retardation.
Key words
Lennox-Gastaut syndrome - Nonconvulsive status epilepticus - Mental retardation - Prognostic factors
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Prof. Dr. med. Rudolf Korinthenberg
Department of Neuropaediatrics and Muscular Disorders Paediatric University Hospital
Mathildenstr. 1
79106 Freiburg
Germany
eMail: E-mail: rudokori@kkl200.ukl.uni-freiburg.de
