- Lennox-Gastaut syndrome
- A severe form of epilepsy that usually begins in early childhood and is characterized by frequent seizures of multiple types, mental impairment, and a particular brain wave pattern (a slow spike-and-wave pattern). The seizures that are notoriously hard to treat and may lead to falls and injuries can be reduced in frequency by treatment with lamotrigine, a chemically novel antiepileptic drug. The syndrome is named for W.G. Lennox and H. Gastaut who described it. The Lennox-Gastaut syndrome usually develops in children between 1 and 8 years of age and is characterized by seizures, developmental delay, and behavioral disturbances such as poor social skills and attention-seeking behavior. Most children with it have impaired intellectual functioning or information processing. The disorder may be caused by brain injury, severe brain infections, genetic brain diseases, or developmental malformations of the brain. In some cases, no cause can be found. The types of seizures, which vary among Lennox-Gastaut patients, include tonic (stiffening of the body, upward deviation of the eyes, dilation of the pupils, and altered respiratory patterns), atonic (brief loss of muscle tone and consciousness, causing abrupt falls), atypical absence (staring spells), and myoclonic (sudden muscle jerks). There may be periods of frequent seizures mixed with brief, relatively seizure-free periods. The prognosis (outlook) for individuals with Lennox-Gastaut syndrome varies. There is no cure for the disorder. Complete recovery including freedom from seizures and normal development is very unusual.
* * *Len·nox-Gas·taut syndrome 'len-əks-gas-'tō- n an epileptic syndrome esp. of young children that is marked by tonic, atonic, and myoclonic seizures and by atypical absence seizures, that is associated with mental retardation, that is prob. caused by various forms of brain damage (as from cerebral hemorrhage, encephalitis, or developmental or metabolic disorder), and that is characterized between seizures by an EEG having a slow spike and wave patternLennox William Gordon (1884-1960)American neurologist. Lennox served for many years on the neurology faculty at Harvard University Medical School. Concurrently he was on the staff of Boston's Children's Hospital, serving as chief of the Seizure Division from 1947. In 1939 he organized the National Epilepsy League, a lay organization devoted to combating societal discrimination against epileptics. He devoted his career to researching epilepsy and migraine, focusing on such topics as blood chemistry, cerebral circulation, the electrical activity of the brain, and metabolism. With Frederic A. Gibbs he demonstrated the value of electroencephalography in the diagnosis and treatment of epilepsy. He is also credited with establishing the effectiveness of trimethadione in the treatment of absence seizures in children. He published numerous books and articles on epilepsy, including Epilepsy and Related Disorders (1960), in which he described Lennox-Gastaut syndrome.Gas·taut gȧs-tō Henri Jean-Pascal (1915-1995)French neurologist. Gastaut served on the medical faculty of Marseilles University, eventually becoming its dean. He also acted as chief neurobiologist for public hospitals in Marseilles. From the early 1960s he was director of the Regional Center for Epileptic Children and director of the neurobiological research unit at the National Institutes of Health. His research centered on electroencephalography and epilepsy. The author of many books, monographs, and articles, he published the first description of what is now known as Lennox-Gastaut syndrome in 1957 in an article coauthored with M. Vigoroux, C. Trevisan, and H. Regis.
* * *Len·nox-Gas·taut syndrome (lenґəks gah-stoґ) [W.G. Lennox; Henri Jean Pascal Gastaut, French biologist, 1915â€“1995] see under syndrome.
Medical dictionary. 2011.