Lennox-Gastaut syndrome


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Len·nox-Gas·taut syn·drome

(len'ŏks gahs-tō),
a generalized myoclonic astatic epilepsy in children, with mental retardation, resulting from various cerebral afflictions such as perinatal hypoxia, cerebral hemorrhage, encephalitides, and maldevelopment or metabolic disorders of the brain; characterized by multiple seizure types (generalized tonic, atonic, myoclonic, tonic-clonic, and atypical absence) and background slowing and slow spike and wave pattern on EEG.
Synonym(s): Lennox syndrome

Lennox-Gastaut syndrome

[len′oks-gästō′]
Etymology: William G. Lennox, American neurologist, 1884-1960; Henri Gastaut, French biologist, b. 1914
a condition in which a variety of generalized seizures, such as tonic, atonic, absence, tonic-clonic, akinetic, and myoclonic, begin to appear in the first 5 years of life. Seizures are often intractable and may require multiple antiepileptic medications. Mental retardation is often present. Among suggested causes are inherited metabolic abnormalities and perinatal or postnatal disorders. Also called Gastaut's disease.

Lennox-Gastaut syndrome

Lennox syndrome Neurology A severe encephalopathic form of epilepsy that constitutes 5% of all childhood epilepsies Clinical Early onset of multiple types–eg, absence, atonic, and others of seizures, slow spike-wave EEG pattern, cerebral atrophy, and often progressive mental retardation; seizures are poorly controlled even with multiple anticonvulsants Prognosis For cognitive development, poor; felbamate may ↓ Sx. See Felbamate.

Len·nox-Gas·taut syn·drome

, Lennox syndrome (len'ŏks gahs-tō' sin'drōm)
A generalized myoclonic astatic epilepsy in children, with mental retardation, resulting from various cerebral afflictions such as perinatal hypoxia, cerebral hemorrhage, encephalitides, maldevelopment or metabolic disorders of the brain; characterized by multiple seizure types (generalized tonic, atonic, myoclonic, tonic-clonic, and atypical absence) and background slowing and slow spike and wave pattern on electroencephalogram; patients are usually mentally retarded or developmentally delayed.

Gastaut,

Henri, French biologist, 1915–.
Gastaut syndrome - juvenile hemiplegic epilepsy with ipsilateral signs.
Lennox-Gastaut syndrome - see under Lennox

Lennox,

William G., U.S. neurologist, 1884-1960.
Lennox-Gastaut syndrome - a generalized myoclonic astatic epilepsy in children, with mental retardation. Synonym(s): Lennox syndrome
Lennox syndrome - Synonym(s): Lennox-Gastaut syndrome
References in periodicals archive ?
The report provides a snapshot of the global therapeutic landscape of Lennox-Gastaut Syndrome
A review of the Lennox-Gastaut Syndrome products under development by companies and universities/research institutes based on information derived from company and industry-specific sources
24 October 2011 - Danish pharma company H Lundbeck A/S (CPH:LUN) said today that the US Food and Drug Administration (FDA) has accepted oral antiepileptic drug Onfi (clobazam) for the treatment of Lennox-Gastaut syndrome (LGS).
The frequency of drop seizures declined significantly for patients with Lennox-Gastaut Syndrome (LGS) who received the highest doses of clobazam in a double-blind, placebo-controlled trial.
The researchers studied 43 patients at Children's Hospital, between the ages of twelve months and 15 years, where majority of the children who responded to the diet had either a severe form of childhood epilepsy called Lennox-Gastaut syndrome or symptomatic generalized epilepsy.
Examples of medicines in development for rare diseases (also known as "orphan drugs") include a monoclonal antibody for the treatment of chronic sarcoidosis, an immune system disorder; a medicine for Lennox-Gastaut syndrome, a severe form of epilepsy; a gene therapy for cystic fibrosis; and a medication for epidermolysis bullosa, a group of inherited disorders characterized by the development of skin blisters in response to minor traumas.
The mechanism of action is not clear, but ketogenic diet has prevented epileptic seizures in many children who did not respond to treatment with anti-epileptic medications, including children with Lennox-Gastaut syndrome.
OVATION Pharmaceuticals has undertaken a clinical development program to gain Food and Drug Administration approval for clobazam for the treatment of pediatric and adult patients with refractory epilepsy, specifically in Lennox-Gastaut syndrome (LGS).
In adults, lamotrigine is approved as adjunctive therapy for the generalized seizures of Lennox-Gastaut syndrome and for conversion to monotherapy in adults with partial seizures who are receiving treatment.
The approved pediatric indications of lamotrigine are management of simple or complex partial seizures or Lennox-Gastaut syndrome, a devastating childhood epileptic encephalopathy.