Why is it important?

Epilepsy occurs more frequently in patients with developmental disabilities than in the general population,1 but the goals of epilepsy therapy are the same:

• the best quality of life with the fewest seizures (none, if possible)
• the fewest treatment side effects
• the fewest possible medications

The obstacles to achieving these goals in patients with developmental disabilities are numerous.1

One of the most important factors in proper management is the recognition of epilepsy syndromes. Classifying patients according to epilepsy syndromes facilitates the identification of relatively homogeneous populations among this heterogeneous group. Identifying a relatively homogeneous population facilitates:

• the selection of appropriate and optimal treatments
• the provision of more accurate prognoses
• the delivery of better family education and counseling

In patients with epilepsy and developmental disabilities, recognition of the epilepsy syndrome called Lennox-Gastaut syndrome (LGS) or childhood epileptic encephalopathy with diffuse slow spike and waves is important for three reasons:

1. LGS is very common, occurring in 7% of children with mental retardation and 16.3% of institutionalized patients.5,6
2. Patients with LGS have an increased risk of death, because of multiple disabilities and generalized-onset seizures.
3. Effective treatments have recently become available, which can improve the patient’s quality of life while reducing seizure frequency (in some cases, producing seizure freedom).

Adapted from: Glauser TA and Morita DA. Lennox-Gastaut syndrome. In: Devinsky O and Westbrook LE, eds. Epilepsy and Developmental Disabilities. Boston: Butterworth-Heinemann; 2001;65–78.
With permission from Elsevier (www.elsevier.com).
Reviewed and revised May 2004 by Steven C. Schachter, MD, epilepsy.com Editorial Board.