In patients with Lennox-Gastaut syndrome (LGS), epilepsy most often begins between the first and seventh years of life and is manifested by tonic, atonic and myoclonic seizures, leading to multiple falls and atypical absence seizures; interictal EEG reveals bilateral, although not necessarily symmetrical, slow (<2,5 Гц) активность пик-волна.
The scope of Lennox-Gastaut syndrome is understood differently by different researchers; different criteria are used both to define the syndrome and to differentiate it from similar syndromes such as myoclonic epilepsies (for a discussion of the problems of the nosology of the syndrome, see Aicardi and Levy Gomes 1988, 1989; Arzimanoglou et al ., 2004).
Currently, most researchers have adopted the definition proposed by Beaumanoir and colleagues (Beaumanoir and Dravet, 1992; Beaumanoir and Blume, 2005), i.e. a syndrome with several types of seizures, with slow peak-wave complexes, spikes and bursts of spikes with a frequency of 10-20 Hz during sleep, most often in combination with mental retardation.
Seizures in Lennox-Gastaut syndrome include "core" seizures (myoatonic and tonic, atypical absences, and nonconvulsive status epilepticus episodes), which may alternate with less characteristic seizures such as tonic-clonic, partial, or unilateral seizures.
Tonic seizures are observed in 55-92% of cases. Clinically, they are manifested by rigidity of the body, mainly of the axial muscles and muscles of the proximal limbs; extension of the trunk, lower and upper limbs occurs more often than flexion. They may be limited to the muscles of the trunk and neck, with opening often accompanied by apnea.
Sometimes they appear only by opening the eyes and weak “stretching” movements. The duration does not exceed 30 seconds, often no more than 10 seconds. Even in these cases, the contraction is clearly tonic, manifested clinically by prolonged muscle contraction and muscle activity on electromyography (Aicardi and Levy Gomes, 1988). Ictal EEG reveals a generalized discharge of fast activity (>10 Hz) with increasing or large amplitude (Beaumanoir and Dravet, 1992; Arzimanoglou et al., 2004; Beaumanoir and Blume, 2005), sometimes followed by several spike-wave complexes. The same discharges of short duration without clinical manifestations are recorded in the slow-wave sleep phase.
Tonic seizures often occur at night, but their manifestations may be so mild that they go unnoticed (Aicardi and Levy Gomes 1988; Yaqub 1993; Beaumanoir and Blume 2005).
A) Atonic seizures occur in 26-56% of patients with Lennox-Gastaut syndrome. They represent a sudden relaxation of muscles and are the usual reason falls in such children, although tonic and myoclonic attacks can also lead to falls. They are serious practical problem, often causing injuries that cannot be prevented by wearing a helmet. The mechanism of falls when it is impossible to perform a polygraphic recording often remains unclear, and in these cases it is better to give preference to the vague term astatic seizure.
Most atonic seizures occur with loss of consciousness, and it is not entirely clear whether they are exclusively atonic or are accompanied by tonic phenomena. In most cases, the same EEG changes are detected as during tonic seizures. This distinguishes them from purely atonic seizures associated with bursts of spike-wave activity observed in myoclonic-astatic epilepsies (Oguni et al., 1993, 1996, 2001a).
b) True myoclonic seizures can occur in almost 28% of cases, they are also accompanied by falls. The type of seizure observed depends on the age of onset of Lennox-Gastaut syndrome. In early onset cases, tonic seizures predominate, whereas myoclonic seizures and absence seizures are more common in late onset cases (Chevrie and Aicardi, 1972, 1996).
Lennox-Gastaut syndrome. EEG manifestations in a child with various types seizures, with a predominance of tonic attacks and atypical absences.An EEG fragment recorded upon awakening shows sharp waves and slow components of about 2-2.5/sec over the anterior half of the head (left).
During sleep, a flash of 10-12/sec spike components associated with spontaneous opening of the eyes is visible (right).
V) Atypical absence seizures present in 17-60% of patients. Although their onset and ending may be more violent than typical absence seizures, they exhibit the same loss of perception and ability to respond with a subtle motor component (stupor, hypotonia, simple automatisms), and their diagnosis depends on the clinical context and accompanying EEG changes . Sometimes during a seizure there are slow spike-wave complexes, difficult to distinguish from interictal spike-waves, but more often fast discharges are detected, the same as in tonic seizures.
d) Episodes occur frequently non-convulsive epistatus(Dravet et al., 1986; Beaumanoir et al., 1988), which can last several days or even weeks. They can cause alternating periods of improvement and deterioration with significant changes in the patient's reaction time and mental activity. The most common type is characterized by alternating tonic attacks and episodes of erratic behavior, often with intermittent myoclonic contractions of the muscles of the face and upper extremities, lasting from several hours to weeks (Arzimanoglou et al., 2004).
Interictal EEG of patients with Lennox-Gastaut syndrome shows a diffuse slow spike-wave pattern, with slow truncated peaks followed by irregular 1-2 Hz slow waves of varying amplitude, usually accompanied by slow, erratic background activity. These EEG manifestations can be asymmetrical and are often accompanied by obvious clinical manifestations. They respond poorly or not at all to hyperventilation or photostimulation, but are activated during drowsiness and slow-wave sleep (Aicardi and Levy Gomes, 1988).
In phases not related to REM sleep, rhythms lasting several seconds with a frequency of 10-20 Hz are characteristic, possibly reflecting subclinical or minimally expressed tonic seizures. In the same phase, instead of slow spike-wave complexes, polyspike-wave complexes often appear.
d) Mental retardation before the onset of seizures is observed in 20-60% of cases (Arzimanoglou et al., 2006). Number of patients who develop over time mental retardation, five years after the onset of the disease increases to 90% (Chevrie, Aicardi 1972). Some patients have a clear loss of skills. Psychotic symptoms are often present.
e) Etiology of Lennox-Gastaut syndrome heterogeneous. Important role Brain damage plays a role, while genetic factors are considered less important. Up to two-thirds of cases may result from detectable brain abnormalities or develop in patients with a history of developmental delay; in the latter case they are considered symptomatic. A significant number of cases of Lennox-Gastaut syndrome develop after infantile spasms, with a gradual transition, and are caused by the same brain lesions as infantile spasms.
However, brain malformations are less common in Lennox-Gastaut syndrome, and Lennox-Gastaut syndrome is extremely rare in Aicardi syndrome and lissencephaly. There are often focal or multifocal anomalies of cortical development, and cases of band heterotopia and bilateral perisylvian syndrome have been reported. The disease can be caused by tuberous sclerosis and more rare neurocutaneous syndromes, such as linear sebaceous nevus and Ito hypomelanosis (cases from personal practice). Acquired destructive lesions are also uncommon. Metabolic disorders are extremely rare, although Lennox-Gastaut syndrome has been described in Leigh encephalomyelopathy (Matsuishi et al., 1985). There are rare cases of Lennox-Gastaut syndrome that developed secondary to brain tumors (Honda et al., 1985).
In some cases it is absent obvious reason diseases, they are considered cryptogenic. Due to the frequency of Lennox-Gastaut syndrome in patients with unilateral lesions, attempts have been made to distinguish between “true” Lennox-Gastaut syndromes and cases of bilateral secondary synchrony (Gastaut and Zifkin, 1988). In fact, cases of secondary bilateral synchronization meet the usual criteria for the diagnosis of Lennox-Gastaut syndrome, although their isolation may be useful from the point of view of the possibility of surgical treatment. PET imaging has identified several metabolic patterns (focal, multifocal or diffuse) that may correspond to different disease mechanisms (Chugani et al., 1987; Iinuma et al., 1987; Theodore et al., 1987). Their practical significance remains unclear because diagnostic criteria for Lennox-Gastaut syndrome vary across studies.
and) Diagnosis of Lennox-Gastaut syndrome does not pose any difficulties when using clear criteria. However differential diagnosis LGS with other fall syndromes can be problematic (for a discussion, see Aicardi, 1996), especially in the early stages of the disease or in cases where tonic seizures are not yet present (Arzimanoglou et al., 2004). A serious concern may be rare cases of “atypical childhood benign epilepsy” (Aicardi and Levy Gomes, 1992) or pseudo-Lennox syndrome (Hahn, 2000), in which recurrent falls and diffuse paroxysmal EEG activity during sleep may be suggestive of Lennox-Gastaut syndrome.
Cases of these diseases can be relatively benign and correct diagnosis it's important.
h) Prognosis for Lennox-Gastaut syndrome unfavorable (Arzimanoglou, 2003). Approximately 80% of patients continue to have seizures and, depending on the nature of the seizures and mental decline, only a few are able to live independently. The outcome is particularly poor in patients with brain damage, early onset or previous infantile spasms, and mental retardation before the onset of seizures. Normal level mental development persists in less than 10% of patients. It seems difficult to differentiate these cases from cases with less favorable outcome, Although late age onset of the disease positive reaction on hyperventilation and a higher incidence of peak-wave complexes with a frequency of 3 Hz have some prognostic significance.
Cases with such signs are referred to as “intermediate petit mal,” but the identification of this subgroup is controversial, since the diagnostic criteria remain the same as for Lennox-Gastaut syndrome in general. In typical cases, the clinical picture changes with age. Between the ages of 15 and 20, the overall frequency of seizures usually decreases. Atypical absence seizures and drop attacks become rare, but all other types of seizures persist, including tonic seizures during sleep. It is likely that parents of older children are less likely to notice tonic seizures during sleep (Arzimanoglou et al., 2004; Beaumanoir and Blume, 2005).
i) Lennox-Gastaut syndrome is difficult to treat treatment. Among modern antiepileptic drugs, combinations of sodium valproate and benzodiazepines have been ineffective, but it is still worth trying to prescribe these drugs to selected patients. Some clinicians report that carbamazepine is effective for partial-onset and tonic seizures, but it has no effect or even worsens other types of seizures. Due to the lack of interaction with other antiepileptic drugs (AEDs), vigabatrin has been used with some success, especially in patients with Lennox-Gastaut syndrome, in whom focal seizures combined with nuclear seizures of this syndrome. Lamotrigine has been reported to be effective (Motte et al., 1997), especially for atonic seizures; this may be the best option at this time, especially in combination with sodium valproate.
Felbamate, used in accordance with current guidelines and with ongoing monitoring, is often effective despite its possible toxicity (Pellock et al., 2006). Topiramate has been reported to be effective in some cases (Sachedo et al., 1999). Interestingly, a combination is often required since individual drugs are only effective against certain types of seizures. Most treatment regimens are based on clinical experience or on data from uncontrolled studies, and only a few of them, including felbamate, topiramate and lamotrigine therapy, are based on controlled studies (Glauser and Morita 2001; Hancock and Cross 2003). Reliable information about the interaction of AEDs is required; Usually such children are treated more effectively by pediatric neurologist- epilepsy specialist. A recent Cochrane review (Hancock and Cross, 2003) also concluded that the optimal treatment for Lennox-Gastaut syndrome has not yet been found, and no studies have shown that any drug is highly effective. Until further research is carried out, clinicians will have to continue to prescribe treatment individually for each patient, weighing the possibility of improvement against the risk of possible side effects.
Because of drug therapy often proves ineffective, it may be worth trying other treatment methods. The ketogenic diet has good short-term effects (Kinsman et al., 1992), despite the practical difficulties caused by its unpalatability and side effects. However, Kinsman and his colleagues were able to extend treatment to a year or two, which controlled seizures in half of the patients. Interestingly, they claim that after two years the diet can be discontinued without causing a recurrence of seizures. Steroids are mainly used for emergency care the patient during the period of severe activity of epilepsy or status. It was proposed intravenous administration immunoglobulins, but this method has not been adequately tested.
Thyrotropin-releasing hormone (Matsumoto et al., 1987) was used mainly in Japan. Also alternative method treatment may be stimulation vagus nerve; Surgical resection is used in some cases of cortical dysplasia. In selected cases, callosotomy may be performed to control seizures with falls, which are the most disabling type of seizure.
What is Lennox-Gastaut Syndrome
Occurs in children aged 2 to 8 years, but more often in preschool age, 2-6 years.
Approximately 30% of such attacks are recruited from cases of West syndrome.
What causes Lennox-Gastaut syndrome?
It refers (like West syndrome) to multifactorial epilepsies, that is, there are suspicions of a symptomatic etiology, but not supported by the results of morphological studies, and the etiology in this case remains cryptogenic. In the International Classification, epilepsy is distinguished in the section Generalized forms of epilepsy as cryptogenic and symptomatic. Organic residual cerebral syndromes (pre-, peri- and postnatal), subacute encephalopathies, neurometabolic diseases, and tuberous sclerosis are often observed.
Symptoms of Lennox-Gastaut Syndrome
Onset at the age of 2 to 8 years, late forms from 10 to 20 years.
The most common types of seizures are myoclonic-astatic seizures, atypical absence seizures, lightning-fast nodding seizures, sudden falls, tonic seizures (usually in sleep). Generalized tonic-clonic, myoclonic, partial seizures. There is a tendency to a series of various attacks with a state of stupor, as well as an imperceptible transition to status epilepticus.
Neurology: in 40% of cases - cerebral paresis and hypotonic-astatic disorders.
Psyche: usually - mental retardation to the extent of severe dementia, psychoorganic disorders. In 80% of cases - severe cognitive and personality disorders organic type.
Neuroradiology and pathomorphology: focal or diffuse structural disorders.
Diagnosis of Lennox-Gastaut Syndrome
Based on typical clinical picture and EEG data. The EEG usually shows background changes in the form of slow peak-waves less than 3 Hz, a series of peaks at night (up to 100 per night), often multifocal changes. Previously, it was believed that the pattern of rhythmic peak-wave complexes of 2.5 Hz was pathognomonic for Lennox-Gastaut syndrome. In fact, the description of the EEG pattern in Lennox-Gastaut syndrome is the same as hypsaremia, only with a greater content of “acute” phenomena. The EEG finding of hypsarrhythmia confirms the diagnosis of Lennox-Gastaut syndrome.
Differential diagnosis
West syndrome.
Forecast
In 75% of cases there is resistance to therapy. It is possible that myoclonic-astatic seizures persist into adulthood and progress to grand mal seizures. Unfavorable prognostic signs are previous organic brain damage or West syndrome, widespread and frequent tonic convulsions, and a tendency to status.
Treatment of Lennox-Gastaut Syndrome
Usually the attacks are difficult to stop. In more than half of the cases, the syndrome develops against the background of previous encephalopathy, but in 40% of cases it appears as if it were primary.
The first choice drugs are Valproate, Ethosuximide. Second choice - Benzodiazepines, ACTH, corticosteroids. In recent years, Vigabatrin and Lamotrigine have become the first choice drugs, which selectively increase the levels of the inhibitory neurotransmitter GABA in the brain.
Which doctors should you contact if you have Lennox-Gastaut Syndrome?
Psychiatrist
Promotions and special offers
Medical news
14.11.2019
Experts agree that it is necessary to attract public attention to the problems cardiovascular diseases. Some are rare, progressive and difficult to diagnose. These include, for example, transthyretin amyloid cardiomyopathy
14.10.2019
On October 12, 13 and 14, Russia is hosting a large-scale social event for free blood clotting testing - “INR Day”. The promotion is dedicated to World Day fight against thrombosis.
07.05.2019
The incidence of meningococcal infection in the Russian Federation in 2018 (compared to 2017) increased by 10% (1). One of the common ways to prevent infectious diseases is vaccination. Modern conjugate vaccines are aimed at preventing the occurrence of meningococcal infection And meningococcal meningitis in children (even the most early age), teenagers and adults.
25.04.2019
The long weekend is coming, and many Russians will go on holiday outside the city. It's a good idea to know how to protect yourself from tick bites. The temperature regime in May contributes to the activation of dangerous insects...
05.04.2019
The incidence of whooping cough in the Russian Federation in 2018 (compared to 2017) increased almost 2 times 1, including in children under the age of 14 years. The total number of reported cases of whooping cough for January-December increased from 5,415 cases in 2017 to 10,421 cases for the same period in 2018. The incidence of whooping cough has been steadily increasing since 2008...
Medical articles
Almost 5% of all malignant tumors constitute sarcomas. They are highly aggressive, rapidly spread hematogenously, and are prone to relapse after treatment. Some sarcomas develop for years without showing any signs...
Viruses not only float in the air, but can also land on handrails, seats and other surfaces, while remaining active. Therefore, when traveling or in public places, it is advisable not only to exclude communication with other people, but also to avoid...
Return good vision and say goodbye to glasses forever contact lenses- the dream of many people. Now it can be made a reality quickly and safely. New opportunities laser correction vision is opened by the completely non-contact Femto-LASIK technique.
Cosmetics designed to care for our skin and hair may actually not be as safe as we think
Lennox-Gastaut syndrome is a form of generalized (widespread) epilepsy, manifested by various seizures, delayed psychomotor development and specific changes in the electroencephalogram (EEG).
This syndrome is often symptomatic, i.e. it is one of the signs of another condition – encephalopathy. The causes of brain damage to a child - and Lennox-Gastaut syndrome first manifests itself in childhood
- - can be:
- congenital malformations;
- infections suffered in utero and in the first days of life;
Unfortunately, sometimes the origin of encephalopathy cannot be determined. Presence in the family various forms epilepsy is present in 2.5–40% of cases. Often another severe disorder evolves into Lennox-Gastaut syndrome neurological disorder- West syndrome. In addition, parents may notice a child's developmental delay even before the first seizures appear. However, there are also cryptogenic forms of the disease (the cause is not clear), when children do not show any abnormalities until the first manifestations of the syndrome - usually at the age of 3-5 years.
Signs
The main manifestations of the symptomatic and cryptogenic forms of Lennox-Gastaut syndrome can be presented in the following table:
What are these symptoms?
Myoclonic spasms– these are short-term, serial, small twitches of the muscles of the limbs, torso, and face. Their distinctive features in Lennox-Gastaut syndrome are a bilateral, symmetrical nature with a predominant involvement of the arm muscles, mainly located in the upper sections and responsible for flexion. If the convulsions began in the lower extremities, the attack will be accompanied by a sudden fall of the child. This type of seizure in children occurs in 11–28% of cases.
Atypical absence seizures look like a sudden short-term (5-30 s) freezing of the child, his “numbness” and are accompanied by a partial impairment of consciousness. It is often very difficult to notice such behavior in a child, especially if changes in consciousness are minimal. In Lennox-Gastaut syndrome, atypical absence seizures can be detected in 17–60% of cases.
Tonic convulsions- These are prolonged muscle contractions accompanied by impaired consciousness. This syndrome is characterized by both the involvement of individual muscle groups (neck, back, upper limbs) and generalized convulsions. In the latter case, children have the following posture: shoulders abducted, forearms bent, fists clenched, legs straightened or in a triple flexion position. With all types of tonic convulsions, disorders of the autonomic system are detected nervous system: increased heart rate - tachycardia, temporary cessation of breathing - apnea, redness or, conversely, a bluish tint to the face, drooling, lacrimation. The incidence of such attacks is 17–92% of cases.
Atonic attacks are short-lived (1–4 s) and represent a sudden relaxation of muscle groups, which is manifested by falls, head nodding (nodding), and bending of the knees. Account for 26–56% of cases.
Another severe manifestation of Lennox-Gastaut syndrome is developmental delay. Unfortunately, cases of maintaining normal intellectual activity with this disease are extremely rare. The main problems in children are associated with a slowdown or complete cessation of absorption new information and skills development. According to some data, half of the patients do not fully possess self-care skills, and a quarter of the patients who have achieved adolescence, there are problems of socialization in the emotional sphere.
In addition to intellectual impairments, autistic character tendencies, lack of attention and perseverance, hyperactivity and frequent mood changes, including a tendency to aggression, are often noted, which disrupts adaptation in society and negatively affects school performance, even with a moderate decrease in intelligence. The eventual level of development of the child is influenced by the frequency of attacks, their severity and the treatment provided. It should be borne in mind that the younger the child is at the time of the onset of Lennox-Gastaut syndrome, the more significant a decrease in intelligence can be expected.
Treatment of Lennox-Gastaut syndrome
Even with timely treatment, the prognosis is always very serious. The main problem is the resistance of this condition to therapy; tonic seizures are especially difficult to correct. Of course, Lennox-Gastaut syndrome needs integrated approach to treatment, which can be carried out using the following methods:
- Surgical. These include operations aimed at removing the organic substrate of the disease - tuberous sclerosis, tumor. In certain cases, callosotomy is effective (dissection corpus callosum). Vagus nerve stimulation is also used.
- Medication. The basis of drug therapy is anticonvulsants wide range actions (valproate, lamotrigine). As a rule, first treatment is carried out in the form of monotherapy, then a transition to polytherapy is possible. As symptoms improve, medications that are effective against specific types of seizures are added. It is also necessary to use drugs that improve metabolic processes in the brain in treatment.
- In addition, children with Lennox-Gastaut syndrome are justified in prescribing such additional methods treatments such as massage, physical therapy, swimming pool exercises.
- Correction of developmental delays together with a psychologist, speech therapist, and teachers.
Unfortunately, the possibilities modern treatment Lennox-Gastaut syndrome can achieve lasting improvement only in 14% of cases or less. Up to 10% of children die in the first 10 years of life. Varying degrees of intellectual and behavioral impairment are diagnosed in almost 100% of cases. Particular attention should be paid to the treatment of attacks accompanied by falls and increasing the severity of epileptic encephalopathy, as the most life-threatening for children and adult patients. With age, the main directions of treatment for Lennox-Gastaut syndrome remain unchanged, however, the need to take medications to reduce side effects (drugs that protect the liver, hematopoietic system, and others) increases.
Consequences
As you get older, the symptoms of Lennox-Gastaut syndrome not only persist, but in the absence of effective treatment they can also progress. Patients are characterized by a persistent decrease in intelligence up to the limitation of the ability to self-care, periodically recurring convulsive attacks. The main goal of treatment is to achieve sustainable improvement and improve the quality of life of patients in the interictal period. In no case should you refuse treatment or unreasonably change the treatment regimen, since Lennox-Gastaut syndrome, with an unfavorable course, can evolve into a more severe severe forms
epilepsy.Epilepsy represents chronic illness
, characterized by repeated, predominantly unprovoked attacks with impairment of motor, sensory, autonomic, mental or mental functions resulting from excessive neural discharges in the gray matter of the cerebral cortex. Epilepsy - one of the most common diseases in neurology , especially in childhood and adolescence. The incidence (the number of cases of newly diagnosed epilepsy - repeated unprovoked seizures - in 1 year) ranges from 41 to 83 cases per 100,000 child population , with a maximum among children of the first year of life - from 100 to 233 cases per 100,000. Prevalence (“cumulative incidence” - the number of patients with active epilepsy per this moment Leningrad region- 3.0 per 1000 of the total population, and in the Republic of Sakha (Yakutia) - 5.5 per 1000 child population [Guzeva V.I., 2007].
It has now been established that epilepsy is not a single disease with different attacks, but is divided into separate forms- epileptic syndromes. Epileptic syndromes characterized by a stable relationship between clinical, electrical and anatomical criteria; vary in response to antiepileptic therapy and prognosis. In this regard, in 1989, the International Classification of Epilepsies, Epileptic Syndromes and Similar Diseases was developed. For the first time, the classification was based on a syndromic principle, and not on division into individual attacks. This classification is well known to practical doctors. It should be noted, however, that over the past 18 years, the imperfection of this classification is clearly visible. And in 2001, the International Commission on Classification and Terminology released a draft new classification epileptic seizures And epileptic syndromes (Epilepsy. - 2001. - V. 42. - N 6. - P. 796-803). This project has not yet received final approval, but is currently recommended for use in clinical practice.
In recent decades Significant progress has been made in the study, diagnosis and treatment of epilepsy. This progress is associated with changes in approaches to the classification of epilepsy and epileptic seizures, with the improvement of methods for diagnosing epilepsy (especially the development of video-EEG monitoring and high-resolution MRI), as well as with the emergence of more than ten new antiepileptic drugs, the creation of which was based on the principles of high efficiency and safety .
Due to progress in the study and treatment of epilepsy, epilepsy is now classified as a curable disease, and in most patients (65-70%) it is possible to achieve cessation of attacks or a significant reduction in their frequency, however, about 30% are cases of epilepsy that are difficult to treat. The persistence of a significant proportion of treatment-resistant epilepsy requires further study of this disease and improvement of methods for its diagnosis and treatment.
Treatment of a patient with epilepsy is a complex and lengthy process, the basic principle of which can be formulated as the desire to achieve maximum effectiveness ( minimizing epileptic seizures or their cessation) and the best possible tolerance of therapy ( minimum side effects). Doctors should be aware of the latest achievements in the field of epileptology, about modern approaches to the diagnosis and treatment of epilepsy. In this case, the maximum effect in treating the patient can be achieved.
However, very much of the treatment of epilepsy depends on effective collaboration between doctor and patient and family members of the patient, from the correct implementation of medical prescriptions, adherence to the regimen, and a positive attitude toward recovery.
This site was created both for doctors and for patients and their loved ones. The purpose of creating the site is to provide doctors with the most complete information in all areas of epileptology, as well as familiarizing patients and their loved ones with the basics of the disease, the principles of its treatment, the requirements for the regimen, the problems faced by the patient and his family members and the possibilities for solving them, as well as obtaining answers to the most important questions about epilepsy .
With deep respect, Professor, Doctor of Medical Sciences, Head of the Clinic of the Institute of Child Neurology and Epilepsy named after St. Luke,
Konstantin Yurievich Mukhin
Lennox-Gastaut syndrome is a generalized epileptic form, in which convulsive seizures occur, psychomotor development is delayed, all changes are visible on the EEG. A child's brain can be damaged due to birth defects development, after undergoing intrauterine infection. Often the syndrome is a consequence of birth trauma.
In some situations, it is difficult to diagnose encephalopathy in time. With Lennox-Gastaut syndrome, there may be neurological disorders that are characteristic of. Parents notice that the child is behind in development even before the first seizures appear. In some cases, a cryptogenic form is isolated, with which in children for a long time No pathological abnormalities until the syndrome manifests itself at 5 years of age.
Causes of Lennox-Gastaut syndrome
1. The disease occurs due to pathological processes in brain development.
2. Hereditary causes.
3. Damage to the nervous system.
4. Birth trauma, asphyxia, low weight of the child.
5. Heavy infection central nervous system – meningitis, encephalitis, rubella, .
Symptoms of Lennox-Gastaut syndrome
1. Myoclonic convulsive state, when they briefly begin to contract facial muscles, torso, limbs. In the syndrome, seizures are bilateral, symmetrical, often involving muscular system hand, which is responsible for flexion. In the event that cramps strike lower limbs, during an attack the child may fall.
2. Atypical absence is short-lived; the child may freeze for a few seconds and have problems with consciousness.
So, it is important to constantly monitor the attacks that occur with Lennox-Gastaut syndrome. If the syndrome is not treated in time, tonic attacks will begin to worsen, and serious intellectual problems will arise. Most often, the disease affects teenage boys; it rarely occurs in girls.
