Both adults and children can suffer from the uncommon and severe kind of epilepsy known as Lennox Gastaut syndrome (LGS). It results in a variety of seizures that are challenging to manage with medicine. It also has an impact on brain growth and function, which can result in behavioral issues and intellectual disabilities. We'll talk about LGS's causes, symptoms, diagnosis, therapy, and prevention in this blog article.
The exact cause of LGS is unknown, but it may be related to genetic factors, brain injuries, or infections. Some of the possible causes or risk factors of LGS include:
Genetic mutations or syndromes, such as tuberous sclerosis, Down syndrome, or Dravet syndrome
Brain malformations or injuries, such as cerebral palsy, hypoxic-ischemic encephalopathy, or head trauma
Brain infections or inflammations, such as meningitis, encephalitis, or West Nile virus
Unknown causes, which account for about 30% to 35% of cases
The symptoms of LGS usually start between the ages of 2 and 6, but they can occur at any age. The main symptoms of LGS are:
Seizures, which are the most common and characteristic symptom of LGS. There are several types of seizures that can occur in LGS, such as:
Tonic seizures, which cause stiffening of the muscles, especially in the arms, legs, and trunk
Atonic seizures, which cause sudden loss of muscle tone, leading to falls or head drops
Absence seizures, which cause brief lapses of awareness or staring spells
Myoclonic seizures, which cause brief jerks or twitches of the muscles
Tonic-clonic seizures, which cause a combination of stiffening and shaking of the body
Focal seizures, which affect only one part of the brain and cause various symptoms, such as tingling, twitching, or hallucinations
Cognitive impairment, which affects the learning, memory, and thinking abilities of people with LGS. Most people with LGS have mild to severe intellectual disability and developmental delays. They may also have difficulties with speech, language, and communication.
Behavioral problems, which affect the mood, personality, and social skills of people with LGS. They may have symptoms such as aggression, irritability, hyperactivity, impulsivity, anxiety, depression, or autism spectrum disorder.
LGS can be difficult to diagnose because its symptoms are similar to other types of epilepsy or neurological disorders. To diagnose LGS, the doctor may perform the following tests:
Medical history and physical examination, which involve asking questions about the symptoms, family history, and medical history of the person with LGS, and checking for any signs of seizures, cognitive impairment, or behavioral problems
Electroencephalogram (EEG), which is a test that measures the electrical activity of the brain using electrodes attached to the scalp. An EEG can show the type, frequency, and location of the seizures, and also reveal a specific pattern of slow spike-and-wave activity that is typical of LGS
Magnetic resonance imaging (MRI), which is a test that uses a strong magnetic field and radio waves to produce detailed images of the brain. An MRI can show any structural abnormalities or damage in the brain that may be causing or contributing to LGS
Genetic testing, which is a test that analyzes the DNA of the person with LGS to look for any genetic mutations or syndromes that may be associated with LGS
Being a chronic and difficult disorder, LGS needs lifetime care and therapy. Reducing the frequency and intensity of seizures, enhancing cognitive and behavioral function, and improving the quality of life for the individual with LGS are the major objectives of therapy. The primary approaches to treating LGS are:
The first-line therapy for LGS is medication. LGS can be treated with a variety of anti-seizure medications, including stiripentol, valproic acid, lamotrigine, topiramate, rufinamide, clobazam, and felbamate. Nevertheless, no single medication can totally manage every kind of seizure in LGS, and the majority of patients require a combination of two or more medications. The medication selection and dose are determined by the patient's specific response and side effects in LGS.
Dietary therapy is a type of treatment where patients are required to adhere to a specific low-carb, high-fat diet, like the modified Atkins diet or the ketogenic diet. By altering the way the brain utilizes energy, this diet may help lessen seizures. But this diet might also have unintended consequences, such constipation, kidney stones, or weight loss, therefore a doctor and a dietician should be there to closely monitor and supervise the patient.
The vagus nerve, which links the brain to the body, can be stimulated electrically by implanting a tiny device beneath the skin of the chest. This procedure is known as vagus nerve stimulation (VNS). By adjusting the brain's activity, the VNS can lessen seizures. But this medication can also have adverse effects like coughing, hoarseness, or dyspnea, and it has to be adjusted and monitored by a doctor on a frequent basis.
The treatment known as deep brain stimulation (DBS) entails implanting electrodes in particular brain regions—such as the thalamus or the anterior nucleus—that are implicated in the production of seizures. The electrodes are attached to a battery-operated apparatus that is positioned beneath the abdominal or chest skin. By either stimulating or suppressing brain activity, the DBS can help lessen seizures. But this treatment can also have negative consequences, such bleeding, infection, or headaches, and it needs to be surgically adjusted and monitored by a physician on a frequent basis.
The procedure known as a corpus callosotomy entails severing or cutting the corpus callosum, a band of nerve fibers that joins the brain's two hemispheres. Because it stops the convulsions from moving from one side of the brain to the other, the corpus callosotomy can help lessen the frequency of seizures. However, this procedure necessitates a hospital stay and rehabilitation period, and it can also have adverse effects including memory loss, vision issues, or personality changes.
There is no sure way to prevent LGS, but some steps that may help reduce the risk or severity of LGS include:
Seeking medical attention promptly if you or your child have a sore throat, fever, or other signs of infection, as some infections can trigger or worsen LGS
Following the doctor’s instructions and taking the prescribed medication regularly and correctly, as this can help control the seizures and prevent complications
Avoiding triggers that may provoke or worsen the seizures, such as stress, sleep deprivation, flashing lights, or loud noises
Maintaining a healthy lifestyle, such as eating a balanced diet, exercising regularly, and getting enough sleep, as this can help improve the overall health and well-being of the person with LGS
Seeking support and counseling from family, friends, or professionals, as this can help cope with the emotional and social challenges of living with LGS
