Refractory Lennox-Gastaut syndrome

What is Refractory Lennox-Gastaut Syndrome?

Refractory Lennox-Gastaut Syndrome (LGS) is a rare and severe form of childhood-onset epilepsy. It is characterized by a wide variety of seizure types, including abnormal neurological functions, such as intellectual disability and developmental delays. It usually begins during early childhood and is difficult to treat, with most children requiring multiple antiepileptic medications to control seizures. LGS is associated with long-term disabilities and significant morbidity.

Symptoms of Refractory Lennox-Gastaut Syndrome

People with refractory LGS may experience many different types of seizures, including:

  • Generalized tonic-clonic seizures
  • Myoclonic seizures
  • Atonic seizures
  • Absence (petit mal) seizures
  • Drop attacks

Other features of the condition include developmental delay, facial dysmorphism, behavior problems, hypotonia (low muscle tone), and other intellectual and medical problems.

Diagnosis of Refractory Lennox-Gastaut Syndrome

Diagnosis of Refractory Lennox-Gastaut Syndrome is based on the person’s clinical history, seizure types, EEG findings, and laboratory test results. The EEG typically shows a pattern of slow spike-and-wave discharges and multiple spike-wave complexes. Laboratory tests to rule out metabolic disorders may be conducted.

Treatment of Refractory Lennox-Gastaut Syndrome

Treatment of Refractory Lennox-Gastaut Syndrome may involve a combination of medical, dietary, and other therapies. Antiepileptic drugs (AEDs) are used to control seizures, and it may be necessary to use multiple drugs. The ketogenic diet, a high-fat, low-carbohydrate diet, has been found to be beneficial for the treatment of LGS. Other treatments may include cognitive behavioral therapy, physical therapy, occupational therapy, speech therapy, and social skills training.

Prognosis of Refractory Lennox-Gastaut Syndrome

The outlook for people with Refractory Lennox-Gastaut Syndrome varies depending on the individual and the severity of the condition. The use of antiepileptic medications and special diets can help reduce the number and severity of seizures. However, the seizures may still prove difficult to control, and the person may experience long-term disability and significant morbidity.