Disemak secara perubatan oleh Panel Perubatan Hello Doktor
Lennox-Gastaut syndrome (LGS) is a severe form of childhood epilepsy syndrome. It is characterised by the triad of multiple drug-resistant seizure types (commonly tonic, atonic and atypical absence seizure), a specific interictal electroencephalographic (EEG) pattern showing bursts of slow spike-wave (SSW) complexes or generalized paroxysmal fast activity (GPFA) and intellectual and cognitive impairment.
Lennox-Gastaut syndrome develops in the first decade of life, commonly between 3 to 5 years of age and mostly before the age of 8 years old. Prevalence of Lennox-Gastaut syndrome is estimated to be between 1 to 2% of all patients with epilepsy and make up between 1 to 10% of all childhood epilepsies. This condition is found to be more common in males than in females but the reason for this male predominance is still unclear.
Manifestation of various types of classical seizures and varying degree of cognitive impairment are the usual signs and symptoms of Lennox-Gastaut syndrome. The common type of seizures associated with Lennox-Gastaut syndrome are:
Along with cognitive problems, many patients with Lennox-Gastaut syndrome have behavioral and psychiatric problems such as:
Affected children may also experience delays in reaching developmental milestones such as sitting, crawling or walking. Children with Lennox-Gastaut syndrome may develop normally before the onset of seizures, and then lose previously acquired skills (psychomotor regression). Due to the fact that seizures associated with Lennox-Gastaut syndrome are usually resistant to treatment, intellectual impairment and learning problems may worsen over time.
The causes of Lennox-Gastaut syndrome can be divided into two groups, namely identifiable and unknown (cryptogenic).
Identifiable causes are due to underlying genetic, structural or metabolic problems and it can be due to any of those factors or a combination of two or more factors. Between 65% to 75% of patients with Lennox-Gastaut syndrome were found to have identifiable causes and they include:
Risk factors associated with these causes include:
As mentioned earlier, the diagnosis of Lennox-Gastaut syndrome is made based on its triad. Therefore, the test of choice to help make the diagnosis is Electroencephalography (EEG). Two patterns of interest to look for in EEG are bursts of slow spike-wave (SSW) complexes and generalized paroxysmal fast activity (GPFA). Either of these two patterns help to form the diagnosis of LGS with the former being the most commonly seen pattern and the later being observed during sleep.
The goal of treatment is to control the seizures in Lennox-Gastaut syndrome which will then, control the severity and frequency of seizure attack and mitigate associated complications (e.g., hypoxia and drop attacks)
Antiepileptic drugs (AED)
Control of seizure can be achieved using AED. The first line AED for Lennox-Gastaut syndrome is Valproic acid, which is usually combined with Lamotrigine and Topiramate.
Ketogenic diet
The ketogenic diet is found to be effective in childhood refractory seizures in specific genetic disorders (e.g., Glut-1 deficiency syndrome). It consists of a high fat, low carbohydrate ratio and up to 50% of patients achieve significant seizure reduction. However, the effectiveness may decline with time and it requires a supportive team and committed parents. The common side effects of the ketogenic diet are constipation, vomiting, abdominal pain, increase appetite, hypercholesterolemia, mineral deficiencies and growth retardation. The use of a low glycemic index diet and modified Atkins diet (containing nuts/seeds, fruits, or dairy products) are also effective in these patients.
Epilepsy surgery
The best surgical option for managing Lennox-Gastaut syndrome is corpus callosotomy which involves cutting through the corpus callosum, the part of the brain which is responsible for connecting the fibres between the two hemispheres of the brain. By reducing the connection between the two hemispheres, the propagation of seizure impulse will be limited. The surgery can be done either partially (cutting through 4/5 or 2/3 of the corpus callosum) or complete callosotomy. Although complete callosotomy resulted in better improvements, partial callosotomy is used to mitigate the side effect associated with complete callosotomy such as decreased speech output, hemiparesis, gait disturbance, and disconnection syndrome.
Aside from corpus callosotomy, vagal nerve stimulation and focal cortical resection are amongst other surgical procedures that are also being done to treat refractory seizures.
If you have any questions, please consult with your doctor to better understand the best solution for you.
Hello Health Group does not provide medical advice, diagnosis or treatment.
Penafian
Hello Health Group tidak menawarkan nasihat perubatan, diagnosis atau rawatan.
Disemak secara perubatan oleh
Panel Perubatan Hello Doktor
Komen
Kongsi komen anda
Ayuh jadi yang pertama untuk memberi komen!
Sertai Kami atau Log Masuk untuk menghantar komen