Lennox-Gastaut syndrome or childhood epileptic encephalopathy is a severe form of epilepsy that is characterized by multiple types of seizures and mental retardation or regression. It begins in childhood, usually between the ages 3 and 5, and the individual is affected by frequent seizures, often during sleep.
It is defined by atypical absence seizures, causing a partial or complete loss of consciousness, characteristic EEG pattern, and behavioral, cognitive, and psychiatric symptoms.
Many individuals are also affected by drop attacks which is a lapse in muscle tone, resulting in falls that could lead to injuries or can be life-threatening. However, the seizures associated with Lennox-Gastaut syndrome are brief, which leads to learning disabilities and intellectual problems in the children suffering from it.1
Causes Of Lennox-Gastaut Syndrome
The conditions that can lead to Lennox-Gastaut syndrome include:2
- Abnormalities in the brain structure or the brain cortex
- Congenital infections
- Infections such as encephalitis or meningitis
- Injury or trauma
- Genetic disorders such as tuberous sclerosis
- Perinatal hypoxia or reduced oxygen supply to the brain before birth
- Problems with blood flow to the brain
Signs And Symptoms Of Lennox-Gastaut Syndrome
The seizures occurring in Lennox-Gastaut syndrome can vary from child to child. The common signs and symptoms seen in the affected children include:3
- Multiple seizure types
- Electroencephalogram (EEG), a test to find problems in the brain’s electrical activity, shows slow-spike wave between seizures and bursts of fast rhythms during sleep
- Cognitive disabilities, learning disabilities often linked to mental retardation, and psychomotor regression
Types Of Seizures Seen In Lennox-Gastaut Syndrome
- Tonic seizures: It is characterized by altered breathing patterns, increased muscle stiffness and muscle tone, sustained muscle contractions, and a brief loss of consciousness. Although they usually occur at night, they can also be seen during the day.4
- Atonic seizures: Also known as drop attacks, it can cause a sudden loss of muscle tone and a sudden loss of consciousness that can result in abrupt falls. These seizures usually lead to injuries due to the falls.
- Atypical absence seizures: Partial or complete loss of consciousness, accompanied by unresponsive staring. They last only for a few seconds and begin and end abruptly.5
- Myoclonic seizures: It is characterized by abnormal movements and the muscles suddenly begin to jerk. Myoclonic seizures and atypical absence seizures sometimes occur simultaneously.
Treatment For Lennox-Gastaut Syndrome
Although it is difficult to treat Lennox-Gastaut syndrome, there are 3 treatment methods used: medication, surgery, and dietary therapy.9
There are no known natural remedies to treat the Lennox-Gastaut syndrome. However, the ketogenic diet is often recommended to treat certain symptoms of the condition. A ketogenic diet is a high fat, low carbohydrate, and controlled protein diet in which the ratio of fat to carbohydrate plus protein ranges from 2:1 to 4:1.10
The ketogenic diet helps reduce seizures and improves attention, comprehension, activity levels, and endurance. However, its effectiveness remains unclear.
Medical practitioners generally prescribe drugs to treat or control the seizures that come with the the Lennox-Gastaut syndrome. These drugs also improve the psychological condition of the patient, depending on the severity of the condition. The prescribed drugs include topiramate, sodium valproate, lamotrigine, and rufinamide.
Sometimes, doctors may offer surgeries that can reduce the severity and frequency of seizures to prevent drop attacks. Unfortunately, it doesn’t influence the seizure source.
- Unlinking the cerebral hemispheres: Doctors use a surgical method called corpus callosotomy, where the nerves connecting both the hemispheres of the brain are cut. This procedure is used to control seizures.11
- Stimulating the vagus nerve: Electrical impulses are used to stimulate a nerve in the body called the vagus nerve which helps to prevent seizures.
Lennox-Gastaut Syndrome cannot be prevented as it may be caused due to genetic problems, abnormalities or injuries. Despite this, it is important that proper treatment is given to the affected person in order to reduce the severity or frequency of seizures.
|↑1, ↑2||Wheless, James W., and Jules EC Constantinou. “Lennox-Gastaut syndrome.” Pediatric neurology 17, no. 3 (1997): 203-211.|
|↑3, ↑4, ↑5||Lennox-Gastaut Syndrome. National Organization for Rare Disorders.|
|↑6||Maganti, Rama, Paula Gerber, Cornelia Drees, and Steve Chung. “Nonconvulsive status epilepticus.” Epilepsy & Behavior 12, no. 4 (2008): 572-586.|
|↑7||Garmel, Gus M., Alan K. Jacobs, and Mark A. Eilers. “Tonic status epilepticus: an unusual presentation of unresponsiveness.” Annals of emergency medicine 21, no. 2 (1992): 223-227.|
|↑8||Bilo, Leonilda, Sabina Pappatà, Roberto De Simone, and Roberta Meo. “The syndrome of absence status epilepsy: review of the literature.” Epilepsy research and treatment 2014 (2014).|
|↑9||Lennox-Gastaut Syndrome. Department of Neurology, Washington University School of Medicine.|
|↑10||Rogovik, Alexander L., and Ran D. Goldman. “Ketogenic diet for treatment of epilepsy.” Canadian Family Physician 56, no. 6 (2010): 540-542.|
|↑11||Corpus callosotomy. Great Ormond Street Hospital Children’s Charity, National Health Services.|