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Lennox-Gastaut Syndrome (LGS) is a severe form of childhood epilepsy, characterized by both developmental delay and several different seizure types. The average onset is three to five years of age, but it may develop any time between ages one and eight.
All children with Lennox-Gastaut Syndrome are affected differently, some more severely than others.
Seizures include several different types, the most common being tonic (stiffening of the body, eyes looking upward, dilated pupils and changes in breathing), atonic (brief loss of muscle tone and consciousness resulting in sudden falls) and atypical absence seizures (staring spells). Myoclonic seizures (sudden muscle jerks) may also occur. The individual may experience from 9 to 70 seizures per day.
Frequently, head drop (the head falls forward) is the first indication of LGS in early infancy. Infantile spasms may also occur at around three to eight months of age. The infant's arms stretch out, the eyes look upward and the head may nod forward. Infantile spasms usually progress to the mixed seizure activity associated with LGS.
Some level of impaired intellectual functioning, developmental delays and behavioral issues (such as poor social skills and attention-seeking behavior) are observed in most children with LGS. Diagnosis of LGS is made in the presence of a mixed seizure pattern, developmental delay or intellectual disability, and/or an abnormal EEG (with slow spike- and-wave discharges).
Adults also show learning disabilities, difficulties with language, loss of skills which were previously developed, and disorganized movement. Only 17% of the adults with long-term LGS are completely independent, and 50% are completely dependent.
Status Epilepticus: About 50% of those with LGS experience status epilepticus (seizures last at least 30 minutes or repeated seizures occur without regaining consciousness). Children experiencing status epilepticus usually show confusion, stupor or poor balance and coordination rather than convulsions. Convulsive status may lead to brain damage if immediate treatment is not provided. Therefore, Valium is often given to control the convulsive status or seizures during status epilepticus. Factors contributing to status epilepticus include sudden withdrawal from medication, illness, infections and fever.
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The known causes of LGS include brain injury associated with pregnancy and birth (such as severe birth trauma), severe brain infections (such as rubella, toxoplasmosis, meningitis or encephalitis), severe head injury, genetic and metabolic brain diseases (such as tuberous sclerosis), rare childhood diseases and developmental malformations of the brain. In about one-third of the cases of LGS, the cause is unknown.
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LGS is one of most difficult forms of childhood epilepsy to treat. For LGS in general, a combination of drugs is often used, such as sodium valproate, felbamate and clobazam. Anticonvulsants are prescribed in accordance with the most frequent type of seizures experienced by each individual. However, complete seizure control is not always achieved since LGS is one of the most drug-resistant forms of childhood epilepsy, and the positive effects of drug treatment can be short-lived.
In addition to drug therapy, other treatment methods may be included. A ketogenic diet (high in fat and low in carbohydrates) may be beneficial. In some cases, surgery may become an option if the individual is not responding to anticonvulsants and has one specific developmental abnormality of the brain. Several children with intractable epilepsy have been helped by Vagus Nerve Stimulation (VNS). This is a mild electrical stimulation of the vagus nerve, which carries information to the brain.
There is no cure for LGS and the prognosis varies. Typically, LGS continues into adulthood. Complete recovery from seizures and achieving normal development is extremely unusual. About five percent of the children with LGS outgrow their seizures by their teenage years, achieving near normal intelligence and abilities.
However, the outlook is not as bright if the following factors are present: LGS is preceded by infantile spasms; the onset of LGS is prior to age three; there is high seizure frequency or frequent status epilepticus; and the EEG shows slow, persistent background rhythm.
At ODTC, we follow the generally accepted methods of treatment, as outlined above. Our treatment team considers the strengths and needs of each child in order to develop an individual treatment plan.
The staff at ODTC recognize that each child is unique with individual strengths and needs. Our priority is always the well being of each child. If we find that ODTC cannot offer the very best care and services for your child, we are happy to help facilitate placement in another facility.
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Feel free to contact us if you have any specific questions or would simply like more information or resources.
E-Mail (please click on the link): Catharine Crockett, Admissions
Phone: 1-317-815-0505
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National Institute of Neurological Disorders and Stroke
National Organization for Rare Disorders
West Syndrome Support Group (infantile spasms)
Epilepsy Foundation
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This information is provided as a general overview only and is not intended as medical advice. This information should not be used to diagnose or treat any health problem, disease or medical condition. It is not provided as a substitute for professional care. If you have any health concern, please consult the health care provider of your choice.
© Copyright 2005 Indiana Developmental Training Center LLC
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