OVERVIEW
Epilepsy (epilepsy) is the transient signs and symptoms resulting from abnormal excessive or synchronized neuronal activity in the brain caused by a variety of reasons. The location of the abnormal discharges varies and their diffusion network is not accessible, and patients have different forms of seizures, sensory, motor, mental, behavioral, consciousness, vegetative symptoms or both. Each clinical seizure process is epileptic. Epileptic syndrome is a type of epilepsy that can include a variety of etiologic factors that produce complex signs and symptoms, and epileptic syndromes can include a variety of seizure types. Characteristics include age of onset, etiologic classification and genetic mechanisms, ictal signs and symptoms, intermittent and ictal EEG forms and natural evolution.
Questions you may be concerned about
What is LGS Epilepsy Syndrome?
LGS epilepsy syndrome, known as Lennox-Gastaut syndrome, is a type of epilepsy commonly seen in children between the ages of 1 and 8 years old, characterized by complex etiology, varied manifestations, frequent seizures, and difficulty in treatment.
The etiology of LGS epilepsy syndrome is complex and varied, and the exact pathogenesis is still unclear; some patients may develop from West syndrome.
The main features of LGS are multiple seizure types, wide-spike-slow complex waves on EEG, and the triad of mental retardation. The most common types of seizures are tonic seizures, atypical catatonic seizures and atonic seizures.
The most common types of epilepsy are tonic seizures, atypical atonic seizures and catatonic seizures. LGS epilepsy syndrome is usually characterized by frequent seizures, which are difficult to control medically.
Causes
The causes of epilepsy syndrome are extremely complex and can be categorized into the following three groups:
1 Genetic factors
Patients presenting with clinical seizures may have a family history of seizures associated with genetic susceptibility factors, and can present with the same clinical manifestations, or even different types of seizures seen in the same specific family line. The number of genes associated with epilepsy is high and molecular genetics is needed to study known or putative defective genes, which may be the basis for diagnosis of the disease. Sporadic patients are also seen.
2 Structural/metabolic changes
Structural lesions are those in which the patient’s brain tissue exhibits significant structural changes, including acquired disorders: stroke, trauma, infection, ischemia and hypoxia; and congenital disorders: malformations, tumors, and mitochondrial disorders.
3 Unknown etiology
The etiology is unknown and may be due to a genetic defect, or may be the result of an unknown, independent disease for which the current limitations of testing do not allow identification of the cause.
Symptoms
Clinical manifestations are episodic, transient, repetitive and stereotyped. Clinical manifestations take a variety of forms and are categorized according to age:
1. Neonatal period
Seizures usually appear within 5 to 7 days after birth, and can also appear in the first month after birth, or even delayed until 3 months after birth, manifested by increased muscle tone, lip and mouth bruising, and one or both limbs twitching. Common types such as Otaeki Park syndrome: usually starts within 2 to 3 months after birth, and may appear as early as 10 days after birth, presenting with tonic-convulsive seizures that occur in bunches and resolve in 5 to 15 seconds. Seizures occur during both waking and sleeping.
2. Infancy
Seizures are short-lived, sometimes difficult to detect, sometimes only eyes rolled up, lips bruised, eyebrows raised, grinning, nodding, blinking, rapid shaking of one or both limbs for a moment, sometimes several times in a row. Twitching of the limbs may also occur in infants, usually appearing from 3 months of age. Seizures can occur during waking hours, and most often occur just after falling asleep, or when about to wake up. In some specific syndromes, children with recurrent seizures may have delayed or regressed psychomotor development. For example, frequent nodding of the head in patients with West syndrome results in significant regression of psychomotor functioning in the child, leading to epileptic encephalopathy.
3. Childhood
There are many types of seizures, for example, childhood catatonic epilepsy can be characterized by sudden onset of impaired consciousness, cessation of movement, sudden end, frequent seizures, up to hundreds of seizures per day; seizures can continue with the original action or self-talk and other automatisms. In addition, such as common sleep seizures, such as benign epilepsy with central-temporal spikes: the child may be manifested as partial facial muscle or ipsilateral limb convulsions, dysarthria, salivation, generally self-limiting course of the disease. In addition, there are also benign occipital lobe epilepsy in children, early-onset vegetative seizures, rag doll status; late-onset visual hallucinations.
4. Adolescence
It can be manifested as disoriented seizures, or sudden rapid twitching of the limbs, objects flying out of the hands, or twitching of the limbs, head and eyes tilted to one side. Seizures are easy to occur in the early morning just after waking up. The prognosis is better. However, there are also cases with poor prognosis, such as progressive myoclonic epilepsy.
5. There are also epilepsies that have no obvious relationship with age.
For example, reflex epilepsy syndrome: a group of epileptic seizures induced by sensory and cognitive stimuli as reflex epilepsy, which can be seen in visual stimuli, thinking, reading, as well as complex mental and emotional processes induced by disorientation, blinking, and even generalized tonic-clonic seizures. However, fever and alcohol withdrawal-induced seizures are not considered reflex epilepsy.
Examination
Video electroencephalography (EEG) is needed to monitor the patient’s intermittent epileptiform discharges, which can help in diagnosis and treatment. If necessary, video-EEG monitoring during seizures should be performed. MRI of the head is performed to observe the patient’s brain structures for abnormal signals, which can help diagnose the cause and guide treatment. Neuropsychological tests should be performed to assess the patient’s growth and developmental status and to determine whether there is any regression of mental function.
Diagnosis
Diagnosis is based on the patient’s age of onset, known or presumed etiology, electro-clinical features including clinical features, symptoms and signs, pre-morbid cognitive and developmental conditions, post-morbid outcome, EEG features, triggering factors, relationship between seizures and sleep, and drug responsiveness.
Treatment
Different epilepsy syndromes require different treatments according to classification. Incorrect classification and treatment are usually ineffective and may lead to exacerbation of the condition. It is important to visit a regular hospital to determine the treatment plan.
1. Medication
Medication includes antiepileptic drugs and non-antiepileptic drugs such as hormones and ACTH. The aim is to prevent recurrent seizures. The medication regimen varies according to the size of the syndrome. If the seizures are rare and self-limiting, the patient can continue to be observed without oral medication for the time being, but he/she needs to bear the risk of having another seizure. At the same time oral medication according to the patient’s electro-clinical characteristics of the use of appropriate drugs, first of all, the use of monotherapy, the drug needs to be a small dose, gradually and slowly increase the dose, to maintain the absence of seizures or seizures significantly reduce the dose shall prevail. Non-antiepileptic drug therapy such as hormone and ACTH for West syndrome.
2. Surgery
Currently, epilepsy surgery includes epileptogenic resection and palliative surgery. For seizures can not be clear epileptogenic foci, suspected epilepsy syndrome can be considered vagus nerve stimulator or corpus callosum resection. If frequent seizures of transient axial muscle tonus are present callosotomy may be used. Generalized seizures, drug-refractory epilepsy can be used vagus nerve stimulator placement.
3. Ketogenic diet
For high-fat, low-carbohydrate, protein and other nutrients appropriate formulated diet, which does not affect the normal growth and development, but at the same time can treat refractory epilepsy.
4. Transcranial magnetic stimulation therapy
It is a painless, non-invasive and green treatment method that utilizes pulsed magnetic fields to act on the central nervous system, altering the membrane potential of cortical nerve cells to produce induced currents, affecting metabolism and neuroelectrical activity in the brain, thus reducing seizures.