Lenox-Gastaut syndrome, or LGS, is a severe form of epilepsy that usually appears in infancy or early childhood. The majority of seizures experienced by affected children include tonic, atonic, and atypical absence seizures. In at least 70 percent of cases, LGS has a cause that can be identified (Wheless, 2020). These patients are usually referred to as having symptomatic Lennox-Gastaut syndrome. On a cellular level, these causes often present as abnormal growth or development in the brain cortex, specifically in cortical dysplasia, congenital infections, and perinatal hypoxia. There are also cases of encephalitis or meningitis infecting the central nervous system. Bacterial or viral forms of the diseases can enter through macrophages and then move to nerve receptors or through spinal cord neurons. Tuberous sclerosis is a genetic cause of LGS, which is very rare, and produced by mutations in the TSC1 or TSC2 genes. These genes are responsible for cell growth, and Tuberous sclerosis leads to changes that can onset tumors and uncontrolled growth.
Lennox-Gastuat syndrome has several different treatments. The first is the application of antiseizure medication with more than one type of medication usually needed. Second, dietary therapy is also essential to the well-being of a patient with LGS as it can reduce the number of seizures. These diets are often ketogenic, have low glycemic index, and changed Atkin diets (Shafer & Wirrell, 2020). Different surgery and devices can also be implemented to improve the health of a patient. A Corpus Callosotomy separates certain connections in the brain between its left and right side, which reduces the frequency of drop attacks, the most injury-inducing symptom of LGS. Vagus Nerve Simulation therapy is used to prevent seizures by sending regular, mild pulses to the brain.
References
Shafer, P., O. & Wirrell, E. (2020). Lennox-Gastaut Syndrome (LGS). Epilepsy Foundation. Web.
Wheless, J., W. (2020). Lennox-Gastaut Syndrome. National Organization for Rare Diseases. Web.