What is Lennox-Gastaut Syndrome?
It manifests itself in children from 2 to 8 years old, but more often in preschool age, 2-6 years.
Approximately 30% of these seizures are recruited from cases of West syndrome.
Causes of Lennox-Gastaut Syndrome
It belongs (like West syndrome) to multifactorial epilepsy, that is, there are suspicions of the presence of symptomatic etiology, but not supported by the results of morphological studies, and the etiology in this case remains cryptogenic. In the International Classification, epilepsy is highlighted in the section Generalized forms of epilepsy as cryptogenic and symptomatic. Organic residual cerebral syndromes (pre-, peri- and postnatal), subacute encephalopathies, neurometabolic diseases, tuberous sclerosis are often observed.
Symptoms of Lennox-Gastaut Syndrome
Beginning at the age of 2 to 8 years, late forms from 10 to 20 years.
The most common types of seizures are myoclonic-astatic seizures, atypical absences, lightning-fasting nodding cramps, sudden falls, tonic seizures (usually in a dream). Often there are also generalized tonic-clonic, myoclonic, partial seizures. There is a tendency to serialization of various seizures with a state of stupor, as well as an imperceptible transition to epileptic status.
Neurology: in 40% of cases – cerebral paresis and hypotonic-astatic disorders.
Mind: usually – mental retardation to the degree of severe dementia, psycho-organic disorders. In 80% of cases, severe cognitive and personality disorders of the organic type.
Neuroradiology and pathomorphology: focal or diffuse structural disorders.
Diagnosis of Lennox-Gastaut Syndrome
Based on typical clinical presentation and EEG data. On the EEG, there are usually changes in the background in the form of slow peak waves below 3 Hz, at night a series of peaks (reaches 100 per night), often multifocal changes. It was previously believed that for Lennox-Gastaut syndrome, the peak-wave pattern of 2.5 Hz is pathognomonic. In fact, the description of the EEG pattern in Lennox-Gastaut syndrome is the same gipsarimia, but with a large content of “acute” phenomena. The EEG conclusion on hypsarrhythmia confirms the diagnosis of Lennox-Gastaut syndrome.
In 75% of cases – resistance to therapy. Perhaps persistent myoclonic-astatic seizures in adulthood, the transition to large convulsive attacks. Adverse prognostic signs – previous organic brain damage or West syndrome, common and frequent tonic convulsions, tendency to status course.
Treatment for Lennox-Gastaut Syndrome
Usually attacks are difficult to stop. In more than half of cases, the syndrome develops against the background of previous encephalopathy, but in 40% of cases it appears as if primary.
First-choice drugs – Valproate, Ethosuximide. The second choice is Benzodiazepines, ACTH, corticosteroids. In recent years, Vigabatrin and Lamotrigine have become the first choice drug, which selectively increase the content of the inhibitory neurotransmitter in the brain – GABA.