Lennox-Gastaut syndrome is the name given to a group of severe epilepsies which occur in childhood. It sometimes follows the onset of seizures in the first year of life, which may include infantile spasms (see West syndrome). The onset, which is usually under the age of five years,may be quite acute, with multiple seizures and loss of skills, or initially as infrequent seizures. Although originally described as having a characteristic EEG (electroencephalogram) pattern of slow spike-wave, the main clinical features of this condition are of a child developing several types of seizures with slowing or regression in development.

The seizures include tonic (generalised stiffening) myoclonic (fast jerks), atypical absences (loss of responsiveness which may not be complete), partial (stiffening and jerking of one body segment) and generalised (stiffening and jerking of the whole body) tonic clonic attacks. Very commonly the seizures also include episodes of non-convulsive status epilepticus in which the child lapses into a groggy state and may have difficulty with communication, feeding and walking. There are more children and young adults with multiple seizure types and regression than those who fit the above EEG definition and from the families' point of view it is reasonable to include all within the group.

This condition is often resistant to drug treatment and may pose long term problems with variable and unpredictable levels of functioning. A small number of children show a milder and self-limiting pattern. The condition may occur in a child with normal or slow early development. Occasionally surgical treatment may be possible if there is removable area of brain damage responsible for the seizures or if drop attacks are the main seizure type.

Most children have learning difficulties which may vary both in extent and from time to time. A range of psychological problems, including attention deficit, and autistic features are common.

Inheritance patterns
None has been found

Prenatal diagnosis
None

Medical text written December 1996 by Professor B Neville. Last updated January 2003 by Professor B Neville, Professor of Childhood Epilepsy, Institute of Child Health, London, UK.

Further Online Resources
Medical texts in The Contact a Family Directory are designed to give a short, clear description of specific conditions and rare disorders. More extensive information on this condition can be found on a range of reliable, validated web sites and links to them are included in the CD-ROM version of this Directory. Further information on these resources can be found in our Medical Information on the Internet article.

LENNOX-GASTAUT SUPPORT GROUP

Andrew Gibson
Lennox-Gastaut Support Group
9 South View
Burrough on the Hill
Melton Mowbray
LE14 2JJ
Tel: 01664 454305
e-mail: andrew.gibson15@btopenworld.com

The original Lennox-Gastaut Support Group went into abeyance in 1995 and was re-established in 1996. It offers support and information by telephone and letter and linking families where possible. It has information available, details on request. The group is in contact with over 200 families and health care professionals.

Group details last confirmed November 2007.