What is Lennox Gastaut Syndrome?
A seizure is considered to be a short period of bursting abnormal electrical activity in a person’s brain. When it recurs over and over, it is medically termed, epilepsy, which is a seizure disorder. The severe form of epilepsy is recognized as Lennox-Gastaut syndrome. It usually starts before a child turns 4-years-old. It’s characteristics are multiple types of epileptic seizures.
Lennox Gastaut Syndrome
Lennox Gastaut Syndrome Symptoms and Signs
The patients diagnosed with Lennox Gastaut syndrome will experience the following signs and symptoms:
Multiple seizure types
Seizures that are experienced may vary between the following:
Considered as the “drop attack” seizure. During this kind of seizure, the child suddenly falls to the ground, which may be mistaken as poor balance or tripping up. It usually happens many times in a day.
An atypical seizure that last from 10 seconds to several minutes. The child usually stares blankly, appears vacant, and manifests rapid blinking or head nodding.
It can be either convulsive or non convulsive type of epilepticus. It can be a life-threatening seizure, lasting for about 30 minutes or longer with a series of seizures. The person does not regain consciousness in between each seizure.
Another common seizure is a tonic seizure. It occurs when the child is asleep at night. It disturbs their sleep but doesn’t wake the child. These seizures also go undetected because of their brevity.
Tonic clonic seizures
The combination of tonic and clonic seizure. In the tonic phase, the child’s body goes stiff, while in the clonic phase, the seizure begins.
Complex partial seizures
A form of a partial seizure. It is when the child loses awareness of the seizure activity. He or she may perform actions such as walking, riding a bike, or talking and may have physical, thought, and sensory disturbances.
Is a seizure that has a jerking (Myoclonic) characteristic of the muscles without the loss of consciousness.
- Cognitive impairment, which is usually a delay in the development of the cognitive aspect and is seen over time.
Lennox Gastaut Syndrome Causes & Risk Factors
70% of children who have the diagnosis of Lennox-Gastaut syndrome are symptomatic and the remaining 30% are cryptogenic or have no underlying etiology. Some of the children reported to have one of the types, have a family background of epilepsy. However, such a syndrome doesn’t have direct evidence that it can be inherited. Those who have symptomatic Lennox –Gastaut syndrome have the following possible causative factors:
- Brain malformations like cortical dysplasia or tuberous sclerosis
- CNS or central nervous system infection
- Metabolic or degenerative nervous system disorders
- Brain injury before, during, or upon birth
Lennox Gastaut Syndrome Diagnosis
In diagnosing the Lennox Gastaut Syndrome, the physician performs the following actions and confirmative diagnostic exams:
- Medical History pertaining to previous seizure or family history of seizure episodes
- Physical Assessment, such as injury that has been caused by a seizure
- Neurological examination is also assessed to determine if the neurological aspect is already affected.
- EEG or Electroencephalography, which records the brain’s electrical activity. It is an important diagnostic exam that shows either normal or abnormal patterns of brain activity. For patients positive with this syndrome, it will appear on the monitor as continuous spike waves whenever a person is in a slow sleep state and it also shows fast rhythms in a diffuse slow background.
- Other testing, such as urine tests or blood specimens, or an test on cerebrospinal fluid through lumbar puncture, may be done when children have no apparent causative factor that causes the seizure. This is done to detect any underlying genetic or metabolic cause.
Lennox Gastaut Syndrome Treatment
With regards to the treatment of Lennox Gastaut Syndrome, the following treatment therapies are usually prescribed:
- Pharmacological medications such as iamotrigine, ethosuximide, sodium valproate, clobazam and phenytoin. Such medication will not cure the disease, but will only manage the seizures.
- Ketogenic diet, which is a low carbohydrate and special high fat diet. A nutritionist and physicians will prescribe to help in seizure management. It increases the ketones in the body, a popular diet for the epileptic persons.
- Surgical Procedure such as vagus nerve stimulation and corpus callosotomy may be considered.
Lennox Gastaut Syndrome Prognosis
The prognosis for patients with Lennox Gastaut Syndrome differs from one person to another. Since there is no specific treatment for this kind of syndrome, the seizure will continue throughout life.
The long term prognosis of a person with this syndrome with regards to intellectual development and seizure control is poor. Their IQ will deteriorate as time goes by and will need assistance from other people as they get older.
They will also have behavioral problems brought about by the antiepileptic drug’s side effects, seizures, and brain dysfunction. Some children will outgrow seizures upon their teenage years, while others will still continue to experience seizure episodes until they reach adulthood. If this occurs, they will be dependent on others. Still, some will develop infections, which will lead to a shorter life expectancy and will lead to an even poorer prognosis.
Lennox Gastaut Syndrome Complications
The complications associated with this kind of syndrome include:
- Injuries from seizure
- Renal, cardiac, or metabolic complications resulting from the Ketogenic diet
- Disconnection syndrome resulting from corpus callosotomy surgical procedure
- Language disorders brought about by the corpus callosotomy procedure
- Motor dysfunction as a result of the corpus callosotomy procedure
- Neurophysical impairment associated with the corpus callosotomy procedure
- Mental retardation as time progresses
Lennox Gastaut Syndrome Prevention
The only preventive action is to prevent epilepic seizures from occurring, specifically the ones that are known to occur with Lennox Gastaut syndrome. You can only prevent seizures from occurring by taking the prescribed medications mentioned above and following a Ketogenic diet or perhaps undergoing a surgical procedure, such as one of the ones mentioned above.
Proofreaded for grammar on 15/10/2012.