Virtual Medical Centre

Lennox Gastaut Syndrome

• What is Lennox Gastaut Syndrome?
• Who gets Lennox Gastaut Syndrome?
• Predisposing Factors
• Progression
• Probable Outcomes
• How is Lennox Gastaut Syndrome Diagnosed?
• How is Lennox Gastaut Syndrome treated?
• Lennox Gastaut Syndrome References
• Drugs/Products Associated with Lennox Gastaut Syndrome

What is Lennox Gastaut Syndrome?

Lennox Gastaut Syndrome (LGS) is a severe form of epilepsy which develops in childhood. Lennox-Gastaut Syndrome is characterised by:

• Multiple seizures (fits) of various types, which are usually difficult to control
• A specific pattern of brain activity on the electroencephalogram (EEG)
• Intellectual impairment.

Who gets Lennox Gastaut Syndrome?

Lennox-Gastaut syndrome is a rare condition. It makes up only 5% of all cases of childhood epilepsy. Lennox-Gastaut syndrome more commonly affects boys. The syndrome usually develops between 3 and 5 years of age, and only rarely develops after the age of 8.

Predisposing Factors

The symptoms of Lennox-Gastaut syndrome are thought to be the result of a non-specific brain injury occurring during a crucial stage of development. In some cases of Lennox-Gastaut syndrome, a predisposing cause can be identified. For example, meningitis, head trauma, or bleeds into the brain may all predispose to LGS. About one quarter of children with Lennox-Gastaut syndrome will have a history of infantile spasms or West syndrome.

Lennox-Gastaut syndrome is not thought to be genetic. However, some patients will have a family history of epilepsy.

Progression

The symptoms of Lennox-Gastaut syndrome usually develop between 3 and 5 years of age. Rarely, patients may develop LGS during adolescence. There are three main features of Lennox-Gastaut syndrome:

1. Multiple types of seizures (fits)
2. Characteristic EEG changes
3. Progressive cognitive (mental) deterioration.

The type of fits can vary amongst the patients. They include:

• Tonic fits (sudden contraction of the muscles)
• Atonic fits (sudden relaxation of the muscles, leading to 'floppy' baby or child)
• Atypical absence fits (blank staring spells)
• Myoclonic fits (sudden jerking movements)

The main type of seizure experienced can change over time. Most young children with Lennox-Gastaut syndrome experience atypical absence seizures, but with age, tonic fits may become more common. Approximately 90% of children with Lennox-Gastaut syndrome will have some intellectual impairment and developmental delay. This often worsens over time. Speech and language may be severely affected. In addition, psychosis, hyperactivity, aggression or autistic features may develop.