Epileptic Encephalopathy, Lennox-Gastaut Type

Alternative Names

  • Encephalopathy of Childhood
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WHO-ICD-10 version:2010

Diseases of the nervous system

Episodic and paroxysmal disorders

OMIM Number

606369

Mode of Inheritance

Autosomal recessive

Gene Map Locus

4q21.3

Description

The Lennox-Gastaut type of epilepsy, although relatively rare in itself, is the most common cause of childhood epilepsies that are difficult to treat. The condition usually presents itself around the age of 3-5 years of age in the form of seizures. The type of seizures differ from patient to patient, and can be atonic-astatic (drop attack), tonic (stiffening), or generalized tonic-clonic (convulsions). The atonic-astatic seizures are the most difficult, because they result in frequent falls, which may cause injuries. Some affected patients go on to develop a non-convulsive status epilepticus, which is a continuous state of seizures, so prolonged that it creates a fixed and lasting condition, and requires medical intervention to end. As children grow older, the type of seizures also changes. The drop attacks ten to cease, and convulsions dominate. One to five of every 100 children with epilepsy are affected with this condition. Boys tend to show a slightly higher incidence of this condition than do girls.

An electroencephalogram (EEG) is the best and most reliable method to diagnose this condition. Treatment is very difficult. Anti-convulsive drugs may have some effect. Other therapeutic strategies include administration of corticosteroids during prolonged seizures, going on a ketogenic diet, and undergoing corpus callostomy or vagus nerve stimulation to combat the drop attacks. Prognosis is generally poor. Only a few of the patients are seizure free by their teenage years, whereas most other patients remain with poor seizure control and intellectual development.

Molecular Genetics

The pathophysiology of Lennox-Gastaut type of epilepsy is not clearly known, although several hypotheses have been made, involving excessive permeability in the frontal areas and immunogenetic mechanisms. Not all cases of Lennox-Gastaut epilepsy are due to genetic defects. However, family history has been noticed in many cases.

A single study found a strong association between this condition and the HLA Class I antigen B7. Similarly, another study found mutations in the JAK3 gene in an affected patient.

Epidemiology in the Arab World

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Other Reports

Oman

Koul (2000) undertook a study to look for patterns of non-convulsive status epilepticus (NCSE) in 12 affected children. Changes in the EEG of continuous or asynchromous 1.5-2 Hz spike and slow wave discharges with background slowing was taken as confirmation for the presence of Lennox Gastaut epilepsy. Three patients (two boys aged 2 and 4-years, and a girl, aged 7 years) were diagnosed with the condition. The two older children had shown tonic clonic and myoclonic seizures before the onset of NCSE. The youngest boy, however, had shown tonic clonic and myoclonic astatic seizures. CT and/or MRI scans in two of the patients were normal, while in the older male, it revealed cerebellar atrophy. All three patients were initially administered an antiepileptic and later put on a benzodiazepine derivative when it failed to control the fragmentary seizures. The outcome remained poor in all three cases.

In 2006, Koul et al. analyzed data from the pediatric neurology department at Sultan Qaboos University Hospital for children undergoing evaluation for developmental delay and epilepsy. Corpus callosum agenesis was the most common neuronal migrational disorder, with 22 patients (55%). There were 14 boys and 8 girls. Eight children (four boys and four girls) in this group had epilepsy. These were infantile spasms in four, generalized tonic-clonic seizures in two, and Lennox-Gastaut syndrome in two.

Saudi Arabia

Yaqub (1993) analyzed electroclinical seizures observed by long video split-screen recording in 21 patients with Lennox-Gastaut syndrome (LGS). All patients had atypical absence seizures, 18 (81%) had tonic seizures, and 4 (21%) had myoclonic-atonic seizures. Tonic seizures were axial with flexion or extension of the head or trunk, or global with generalized tonic spasm mimicking infantile spasm, or involved the eyeballs only (either brief, with upward deviation of eyeballs or long, with oscillatory nystagmus). EEG showed either a bilateral 10-13-Hz rhythm or generalized synchronous spike wave at 3 Hz. Myoclonic-atonic seizures involving limbs, trunk, or neck were either brief or massive; the discharges were 2-3.5-Hz spike wave. Atypical absence seizures evolved gradually, terminated abruptly, and manifested alone or with subtle motor activity or oral automatism. EEG discharges were variable and of different types: (a) Diffuse irregular spike wave at 2-2.5 Hz with or without fragmentation (consciousness was regained during fragmentation or when spike wave discharges were < 2 Hz), (b) irregular diffuse fast activity at 10-13 Hz, or (c) a combination of fast spike wave or sharp waves of increasing amplitude followed by synchronous spike wave discharges at 3 Hz.

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