Idiopathic generalized and focal epilepsy

Convulsive disease, epilepsy, sacred, lunar - as soon as the disease was not called, it manifests itself in frighteningly unexpected periodic seizures, during which patients suddenly fall to the floor, shaking in convulsions. We will talk about epilepsy, which modern medicine considers as a chronic progressive neurological disease, a specific symptom of which is unprovoked, regularly recurring seizures, both convulsive and non-convulsive. As a result of the disease, special personality changes can develop, leading to dementia and complete detachment from the ongoing life. Even the ancient Roman physician Claudius Galen identified two types of the disease: idiopathic epilepsy, that is, hereditary, primary, the symptoms of which appear already at an early age, and secondary (symptomatic), which develops later, under the influence of any specific factors. [1]

In the updated International Antiepileptic League classification, one of the six identified etiological categories of the disease is genetic - an independent primary disease, suggesting the presence of a hereditary predisposition or the presence of genetic mutations that have arisen de novo. Essentially - idiopathic epilepsy in the previous edition. In this case, the patient did not have organic lesions of the brain structures that could cause recurrent epileptic seizures, and in the interictal period, neurological symptoms are not observed. Among the known forms of epilepsy, idiopathic is the most favorable prognostically. [2],  [3], [4]

Epidemiology

An estimated 50 million people worldwide have epilepsy, most of whom do not have access to medical care. [5],  [6] A systematic review and meta-analysis of studies worldwide have shown that the point prevalence of active epilepsy was 6.38 per 1,000 people, and the lifetime prevalence was 7.6 per 1,000 people. The prevalence of epilepsy did not differ between sexes and age groups. The most common are generalized seizures and epilepsy of unknown etiology. [7], [8]

On average, 0.4 to 1% of the world's population requires antiepileptic treatment. The morbidity statistics of developed countries annually record from 30 to 50 new cases of epileptic syndromes per 100 thousand inhabitants. It is assumed that in countries with low levels of development this figure is twice as high. Among all forms of epilepsy, the incidence of idiopathic epilepsy is 25-29%. [9]

Causes of the idiopathic epilepsy

The disease manifests itself in the overwhelming majority of cases in children and adolescents. Patients have no history of previous illnesses and injuries that caused brain damage. Modern methods of neuroimaging do not determine the presence of morphological changes in cerebral structures. The cause of idiopathic epilepsy is considered to be a genetically inherited predisposition to the development of the disease (epileptogenicity of the brain), and not direct inheritance, just among the relatives of the patient, cases of the disease are more common than in the population. [10]

Cases of familial idiopathic epilepsy are rare; monogenic autosomal dominant transmission is currently identified for five episyndromes. Genes were identified whose mutation causes benign familial neonatal and infantile seizures, generalized epilepsy with febrile seizures, focal - frontal with nocturnal seizures and hearing impairment. With other episyndromes, a tendency to develop a pathological process is presumably inherited. For example, to synchronization in all frequency ranges of activity of brain neurons, called epileptic, that is, having an unstable potential difference in an unexcited state on the inner and outer sides of its membrane. In a state of excitement, the action potential of an epileptic neuron significantly exceeds the norm, which leads to the development of an epileptic seizure, as a result of the repetition of which the cell membranes of neurons are more and more affected and pathological ion exchange is formed through destructed neuronal membranes. It turns out a closed cycle: epileptic seizures as a result of repeated hyperintense neural discharges lead to deep metabolic disturbances in the cells of the brain substance, contributing to the development of the next seizure. [11]

A specific feature of any epilepsy is the aggressiveness of epileptic neurons towards the still unchanged cells of the medulla, contributing to the diffuse spread of epileptogenicity and generalization of the process.

In idiopathic epilepsy, in most patients, generalized seizure activity is observed, a specific epileptic focus is not detected. Several types of focal idiopathic epilepsy are currently known. [12]

Studies in juvenile myoclonic epilepsy (CAE) identified chromosomes 20q, 8q24.3, and 1p (CAE was later renamed juvenile absence epilepsy). Studies in juvenile myoclonic epilepsy have shown that the BRD2 susceptibility polymorphisms on chromosome 6p21.3 and Cx-36 on chromosome 15q14 are associated with increased susceptibility to JME. [13],  [14],  [15]  However, the genetic mutation is rare when a person is diagnosed with epilepsy.

Risk factors

Risk factors for the development of the disease are hypothetical. The main one is the presence of close relatives who suffered from epilepsy. In this case, the likelihood of getting sick immediately doubles, or even quadruples. The full pathogenesis of idiopathic epilepsy remains to be seen. [16]

It is also assumed that the patient may inherit the weakness of the structures that protect the brain from overexcitation. These are the segments of the pons varoli, the wedge-shaped or caudate nucleus. In addition, the development of the disease in a person with an inherited tendency can provoke a systemic metabolic pathology leading to an increase in the concentration of sodium or acetylcholine ions in the neurons of the brain. Generalized seizures of epilepsy can develop against the background of a deficiency of B vitamins, in particular, B6. Epileptics have a tendency to neurogliosis (according to pathological studies) - a diffuse overgrowth of glial elements that replace dead neurons. Other factors are periodically identified that provoke increased excitability and the appearance of convulsive readiness against its background.

The risk factor for the development of genetic, as it will now be called, epilepsy is the presence of mutant genes that provoked the pathology. Moreover, a gene mutation does not have to be inherited, it may appear for the first time in a particular patient, and it is believed that the number of such cases is growing.

Pathogenesis

The mechanism of development of idiopathic epilepsy is based on genetically determined paroxysmal reactivity, that is, the presence of a community of neurons with impaired electrogenesis. Any external damaging influences are not detected, as well as the events that became the trigger for the appearance of seizures. Nevertheless, the manifestation of the disease occurs at different ages: in some - from birth, in others - in early childhood, in others - in adolescence and adolescence, therefore, some aspects of pathogenesis at the present stage, apparently, still remain unknown.

Symptoms of the idiopathic epilepsy

The main diagnostic sign of the disease is the presence of epileptic seizures, both convulsive and non-convulsive. Without them, all other symptoms, for example, a characteristic electroencephalogram, history, cognitive and psychological characteristics of the patient are not sufficient to establish the diagnosis of epilepsy. The manifestation of the disease is usually associated with the first seizure, when it comes to epilepsy, this is the most accurate definition. An attack is a more general name that implies an unexpected sharp deterioration in the state of health of any origin, a seizure is a special case of an attack caused by a transient dysfunction of the brain or its part.

Epileptics can experience various disorders of neuropsychic activity - large and small seizures, acute and chronic mental disorders (depression, depersonalization, hallucinations, delirium), stable personal transformations (lethargy, detachment).

Nevertheless, I repeat, the first signs that make it possible to diagnose epilepsy are seizures. The most impressive and impossible to miss attack of idiopathic epilepsy is its generalized manifestation - a large convulsive seizure. I will make a reservation right away that all the components of the symptom complex, which will be described below, are not required even for the generalized form. In a particular patient, only part of the manifestations can be observed.

In addition, usually on the eve of a seizure, its harbingers appear. The patient begins to feel worse, for example, his heart rate increases, his head hurts, unmotivated anxiety develops, he may become angry and irritable, agitated or depressed, gloomy and silent. On the eve of a seizure, some sufferers spend the night awake. Usually, over time, the patient can already guess about the approach of a seizure in his condition.

The formation of an epileptic seizure is directly subdivided into the following stages: aura, tonic-clonic seizures, confusion.

The aura already refers to the onset of the seizure and can be manifested by the appearance of all sorts of sensations - tingling, soreness, warm or cold touches, a slight breeze in different parts of the body (sensory); flashes, glare, lightning, fire before the eyes (hallucinatory); sweating, chills, hot flashes, dizziness, dry mouth, migraine, cough, shortness of breath, etc. (vegetative). The aura can be manifested by motor automatisms (motor) - the patient breaks off to run somewhere, begins to rotate around his axis, wave his arms, scream. Sometimes one-way movements are made (left arm, leg, half of the body). The psychic aura can manifest itself in attacks of anxiety, derealization, more complex than in hallucinatory, auditory, sensory or visual hallucinations. There may not be an aura at all.

Then the second stage immediately develops - the seizure itself. The patient loses consciousness, his body musculature completely relaxes (atony), he falls. The fall occurs unexpectedly for others (the aura often remains invisible to them). Most often, a person falls forward, somewhat less often - backward or on the side. After the fall, the phase of tonic tension begins - the muscles of the whole body or some part of it tenses, becomes enslaved, the patient stretches, his blood pressure rises, his heart rate increases, his lips turn blue. The stage of muscle tone lasts about half a minute, then rhythmic continuous contractions occur - the tonic phase is replaced by a clonic phase - intensifying intermittent chaotic movements of the limbs (more and more abrupt flexion-extension), head, facial muscles, and sometimes eyes (rotation, nystagmus). Jaw cramps often lead to tongue biting during a seizure, a classic epilepsy that is known to almost everyone. Hypersalivation is manifested by foam at the mouth, often stained with blood when biting the tongue. Clonic spasms of the muscles of the larynx lead to sound phenomena during a seizure - mooing, moans. During a seizure, the sphincter muscles of the bladder and anus often relax, resulting in involuntary urination and bowel movements. Clonic seizures last one or two minutes. At the time of seizures, the patient has a lack of skin and tendon reflexes. The tonic-clonic stage of the seizure ends with gradual muscle relaxation and the extinction of seizure activity. At first, the patient is in a state of dim consciousness - some disorientation, difficulty in communication (speaks with difficulty, forgets words). He still has a tremor, some muscles twitch, but gradually everything returns to normal. After a seizure, the patient feels completely overwhelmed and usually falls asleep for several hours, upon awakening, asthenic manifestations also remain - weakness, malaise, bad mood, vision problems.

Idiopathic epilepsy can also occur with minor seizures. These include absences, simple or typical. Complex atypical absences are not common in idiopathic epilepsy. Typical - generalized short-term seizures, during which the patient freezes with a fixed gaze. The duration of the absence is usually no more than a minute, at this time the patient's consciousness turns off, he does not fall, but drops everything he holds in his hands. Does not remember the attack, often continues the interrupted lesson. Simple absences occur without an aura and clouding of consciousness after an attack, usually accompanied by cramps of the facial muscles, mainly involving the eyelids and mouth and / or oral automatisms - smacking, chewing, licking the lips. Sometimes there are non-convulsive absences so short-term that the patient does not even notice them. Complains that his eyes suddenly darkened. In this case, an object dropped from the hands may be the only evidence of an epileptic seizure.

Propulsive seizures - nodding, pecking, “salam-seizures” and other forward movements of the head or the whole body are caused by a weakening of the posotonic muscle tone. At the same time, patients do not fall. Basically, they are found in babies up to four years of age, more often in boys. Typical for nocturnal attacks of the disease. At a later age, they are replaced by large epileptic seizures.

Myoclonus - rapid reflex muscle contractions, manifested by twitching. Cramps can occur throughout the body or only affect a specific muscle group. An electroencephalogram taken at the time of a myoclonic seizure shows the presence of epileptic discharges.

Tonic - prolonged contractions of a muscle group or completely of the entire musculature of the body, in which a certain position is maintained for a long time.

Atonic - fragmentary or complete loss of muscle tone. Generalized atony with a fall and loss of consciousness is sometimes the only symptom of an epileptic seizure.

Seizures are often mixed - absence seizures are combined with generalized tonic-clonic seizures, myoclonic with atonic seizures, etc. Non-convulsive forms of seizures can occur - twilight consciousness with hallucinations and delusions, various automatisms and trances.

Forms

The vast majority of cases related to idiopathic epilepsy manifest in childhood and young age. This group includes epileptic syndromes, mostly relatively benign, that is, well amenable to therapy or not requiring it at all and passing without consequences for the neurological status, which is normal outside of seizures. Also, in terms of their intellectual development, children do not lag behind healthy peers. They record the preservation of the basic rhythm on the electroencephalogram, and modern neuroimaging methods do not detect structural abnormalities of the brain, although this does not mean that in fact they do not exist. Sometimes they come to light later, and it is not yet clear whether they were "overlooked", or whether they provoked the appearance of seizures.

Idiopathic epilepsy has an age-dependent onset and a generally favorable prognosis. But sometimes one form of the disease is transformed into another, for example, childhood absence epilepsy into juvenile myoclonic epilepsy. The likelihood of such transformation and seizures at a later age increases in those children whose close relatives also suffered in childhood and adulthood.

The types of idiopathic epilepsies are not clearly marked, there are discrepancies in the classifiers, some forms do not have strict diagnostic criteria, such as childhood absence epilepsy.

Idiopathic generalized epilepsy

The earliest form of the disease - benign familial and nonfamilial neonatal / infant seizures - is found in full-term newborns literally as early as the second or third day after birth. Moreover, children, in the main, were born by women who quite safely endured and gave birth without significant complications. The average age of development of family forms is 6.5 months, of non-family forms - nine. Currently, genes have been identified (the long arm of chromosomes 8 and 20), the mutation of which is associated with the development of the familial form of the disease. There are no other provoking factors other than a family history of seizures. In an infant with this form of the disease, there are very frequent (up to 30 per day) short, one-two-minute seizures, generalized, focal or with the addition of focal tonic-clonic seizures, accompanied by episodes of apnea. [17]

Idiopathic myoclonic epilepsy of childhood manifests itself in most patients from the age of four months to three years. It is characterized only by myoclonus with preservation of consciousness, manifested by a series of propulsions - rapid nodding movements of the head with abduction of the eyeballs. In some cases, cramps spread to the muscles of the shoulder girdle. If the propulsive seizure begins while walking, it leads to a lightning fall. The onset of a seizure can be provoked by a sharp sound, unexpected and unpleasant touch, interruption of sleep or awakening, in rare cases - rhythmic photostimulation (watching TV, turning on / off the light).

Childhood epilepsy with myoclonic-atonic seizures is another form of generalized idiopathic (genetic) disease. Age of manifestation from ten months to five years. Most develop generalized seizures immediately, lasting 30-120 seconds. A specific symptom is the so-called "blow to the knee", a consequence of myoclonus of the extremities, propulsive nodding movements of the trunk. Usually consciousness is preserved during the seizure. Myoclonus with an atonic component is often accompanied by typical absences, during which consciousness turns off. Absences are observed in the morning after waking up, have a high frequency, and are sometimes supplemented by a myoclonic component. In addition, about one third of children with generalized myoclonic-atonic epilepsy also develop partial motor seizures. In this case, the prognosis worsens, especially in cases where they are observed very often. This may be a sign of the development of Lennox-Gastaut syndrome.

Generalized idiopathic epilepsy in children also includes absence forms of the disease.

Early childhood abscess epilepsy manifests itself in the first four years of life, and is more common in male children. It manifests itself mainly in simple absences. In about 2/5 of cases absences are combined with myoclonic and / or astatic components. In 2/3 of cases, the disease begins with generalized tonic-clonic seizures. Children may have some developmental delays.

Pyknolepsy (childhood absence epilepsy) first appears most often in five to seven-year-old children, girls are more susceptible. It is characterized by a sudden shutdown of consciousness or significant confusion for a time from two to 30 seconds and a very frequent repetition of seizures - maybe about a hundred per day. The motor manifestations of seizures are minimal or absent altogether, but if an aura precedes typical absences and there is a post-seizure clouding of consciousness, then such seizures are referred to as pseudoabsances.

With pyknolepsy, atypical absences with various components can develop - myoclonus, tonic convulsions, atonic states, sometimes automatisms are observed. Various events can stimulate an increase in the frequency of seizures - an unexpected awakening, intense breathing, a sharp change in illumination. In a third of patients in the second or third year of the disease, generalized convulsive seizures may join.

Juvenile absence epilepsy develops in adolescence and adolescence (from nine years to 21 years), begins with absences in about half of cases, and can debut with generalized seizures, which often occur at the time of sleep interruption, awakening or going to sleep. The frequency of seizures is one every two or three days. Hyperventilation is a stimulating factor for the development of absence. Absorption states are accompanied by twitching of the facial muscles or pharyngeal and oral automatisms. In 15% of patients, close relatives also suffered from juvenile absence epilepsy. 

Separately, epilepsy with myoclonic absences (Tassinari syndrome) is distinguished. Manifests from one to seven years, is characterized by frequent absences, especially in the morning, combined with massive muscle contractions in the shoulder girdle and upper extremities (myoclonus). Photosensitivity is not typical for this form; hyperventilation is a provocation of the onset of an attack. In half of the sick children, neurological disorders are observed against the background of hyperactive behavior and a decrease in intelligence.

Idiopathic generalized epilepsy in adults accounts for about 10% of all adult epilepsy. Experts believe that such diagnostic findings in patients over 20 and even 30 years old are the result of late diagnosis due to the ignorance of absences and myoclonic seizures in childhood by patients and their relatives, the relapse of which occurred over a long period of time (more than 5 years). It is also assumed that, very rarely, there may be an unusually late manifestation of the disease.

Misdiagnosis and related inadequate therapy, resistance to adequate therapy for seizures, relapses of idiopathic epilepsy after treatment cancellation were also named as the causes of late manifestations of the disease.

Idiopathic focal epilepsy

In this case, the main and often the only symptom of the disease is partial (localized, focal) epileptic seizures. In some forms of the disease, genes are mapped with which each is associated. These are idiopathic occipital epilepsy, partial with affective seizures,  familial temporal lobe  and essential reading epilepsy.

In other cases, it is only known that localized idiopathic epilepsy occurs as a result of gene mutations, however, the exact responsible genes have not been established. These are autosomal dominant nocturnal fronto-lobe epilepsy and focal epilepsy with auditory symptoms.

The most common localized disease occurs in the form of Rolandic epilepsy (15% of all cases of epilepsy, manifested before 15 years of age). The disease manifests itself in children from three to 14 years of age, its peak falls on 5-8 years. A characteristic diagnostic sign - the so-called "Rolandic peaks" - complexes on the electroencephalogram, recorded in the intraictal (interictal) period. They are also called benign epileptic paroxysms of childhood. Localization of epileptic foci in this form of epilepsy in the perirolandic region of the brain and its lower parts. Rolandic epilepsy in most cases develops in children with a normal neurological status (idiopathic), but symptomatic cases are not excluded when organic lesions of the central nervous system are found.

In the vast majority of patients (up to 80%), the disease manifests itself mainly in rare (twice or three times a month) simple focal seizures that begin in a dream. When waking up or having a seizure in the daytime, patients note that it begins with a somatosensory aura - unilateral paresthesias covering the oral cavity (tongue, gums) or pharynx. A focal seizure then develops. Convulsive contractions of the facial muscles occur in 37% of cases, the muscles of the mouth and pharynx - in 53%, accompanied by severe hypersalivation. In a dream, patients have vocalizations - gurgling, rumbling sounds. In a fifth of patients, muscle contractions involve the muscles of the shoulder and arm (brachiofascial seizures), even half as often they can spread to the lower limb (unilateral). Over time, the localization of muscle contractions can change - to move to the other side of the body. Sometimes in about a quarter of cases, more often in young children, secondary generalized seizures develop during sleep. Up to the age of 15, 97% of patients experience complete therapeutic remission.

Idiopathic occipital epilepsy with late onset (Gastaut type) is much less common. It is a separate disease, manifesting between three and 15 years of age, with a peak around the age of eight. Non-convulsive seizures often occur, expressed in elementary visual hallucinations, which develop quickly and last from a few seconds to three minutes, more often in the daytime or upon awakening. On average, the frequency of a seizure is once a week. The patient in the overwhelming majority of cases does not come into contact in a paroxysmal state. Seizures can progress with the appearance of symptoms such as blinking, illusion of pain, blindness. Vomiting is rare. May be accompanied by headache. Some develop complex visual hallucinations, other symptoms, and a secondary generalized seizure. By the age of 15, 82% of patients diagnosed with Gastaut syndrome experience therapeutic remission.

It also stands out as a variant of the previous form of Panayotopoulos syndrome. It occurs ten times more often than the classic Gastaut syndrome. Idiopathic occipital epilepsy of this type can be of early onset. The peak of manifestation falls at the age of 3-6 years, but the syndrome can develop in both a one-year-old child and an eight-year-old. Moreover, the greatest risk of recurrent seizures is associated with an earlier debut. It is assumed that some cases are not diagnosed, since the seizures are mostly vegetative, with vomiting being the dominant symptom. The child's consciousness is not disturbed, he complains of poor health and severe nausea, which is resolved by severe vomiting with other manifestations up to clouding of consciousness and convulsions. Another form of seizures of Panayotopoulos syndrome is syncope or fainting. Fainting proceeds with tonic or myoclonic components, sometimes with urinary and fecal incontinence, ending in a state of asthenia and sleep. The seizures last from half an hour to seven hours, usually beginning at night. The frequency is small. Sometimes during the entire time of the disease, only one seizure occurs. In 92% of patients, remission of Panayopulos syndrome is noted up to 9 years.

Benign childhood epilepsy with affective seizures (Dall-Bernardine syndrome) is also thought to be a variant of occipital or rolandic epilepsy. The debut is registered from two to nine years. Seizures look like attacks of horror, crying, screaming with manifestations of pallor, increased sweating, salivation, abdominal pain, automatisms, confusion. Seizures often develop during sleep, immediately after falling asleep, but can occur during the day. They arise spontaneously, during a conversation, or any activity without visible stimulation. In most cases, remission occurs before the age of 18.

The above forms of partial idiopathic epilepsy manifest only in childhood. The rest can develop at any time.

Photosensitive localized idiopathic epilepsy is a manifestation of the occipital epilepsy. Seizures are identical to spontaneous, can be supplemented by autonomic symptoms and sometimes develop into secondary generalized tonic-clonic seizures. The factor provoking their onset is the frequent flashing of light, in particular, seizures often occur during video games or watching TV. Manifest from 15 months to 19 years.

Idiopathic partial epilepsy with auditory symptoms (lateral temporal lobe, familial) begins with the appearance of an aura with audiophenomenon. The patient hears blows, rustling, hissing, ringing, other obsessive sounds, complex auditory hallucinations (music, singing), against which a secondary generalized seizure may develop. Manifestation occurs between the ages of three and 51. A characteristic feature of this form is not frequent attacks and a favorable prognosis.

Idiopathic partial epilepsy with pseudo-generalized seizures, which are atypical absences, atonic seizures, and eyelid myoclonus in combination with partial motor seizures, may resemble epileptic encephalopathies on an electroencephalogram. But in children, there is no neurological deficit, and neuroimaging methods do not reveal structural defects.

There is also a genetically determined familial autosomal dominant frontal epilepsy with nocturnal paroxysms. The spread of onset time is very large, seizures can develop from two to 56 years, its exact prevalence is unknown, but the number of families is growing worldwide. Hypermotor seizures occur almost every night. Their duration ranges from half an hour to 50 minutes. Often clonic convulsions join, the patients, coming to their senses, find that they are lying on the floor or in an unusual position or place. At the time of the seizure, a sharp awakening occurs, consciousness is preserved, after the seizure, the patient again plunges into sleep. The onset of a seizure is always associated with sleep - before, during or after. The seizures are usually lifelong and become less pronounced in old age.

Reading epilepsy (graphogenic, speech-induced), a rare type of idiopathic. The debut will take place in late adolescence (12-19 years), among adolescent boys it is much more common. The seizure begins shortly after the beginning of reading, writing, speaking - speech, not only written, but also oral, is a provocative stimulus. Short myoclonus occurs, in which the muscles of the mouth and larynx participate. If the patient continues reading, the seizure often develops further into generalized tonic-clonic seizures. In rare cases, visual hallucinations may join. There may be long-term seizures with impaired speech function. If the patient's behavior is structured correctly, then severe seizures do not develop. Prognostically favorable form.

Complications and consequences

Idiopathic age-related epilepsy generally responds well to treatment, and sometimes does not require it at all and goes away without consequences. However, ignoring its symptoms and hoping that the disease will stop itself is not worth it. Epileptiform activity, especially in childhood and adolescence, when the maturation of the brain and the formation of personality takes place, is one of the reasons for the development of some neurological deficit, leading to a deterioration in cognitive abilities and complicating social adaptation in the future. In addition, in a number of patients, seizures are transformed and are observed already in adulthood, significantly reducing their quality of life. Such cases are associated with both hereditary predisposition and early termination of treatment or its absence.

In addition, in childhood, epileptic encephalopathy can also manifest, the symptoms of which often at the initial stage resemble benign idiopathic forms. Therefore, a thorough examination of the patient and subsequent treatment is an urgent need.

Diagnostics of the idiopathic epilepsy

The diagnostic criterion for this disease is the presence of epileptic seizures. In this case, the patient should be comprehensively examined. In addition to a thorough collection of anamnesis, not only of the patient himself, but also of the family, laboratory and instrumental studies are carried out. It is currently impossible to diagnose epilepsy by laboratory methods, but clinical tests to clarify the general health of the patient are required.

Also, to find out the origin of the seizures, instrumental diagnostics are prescribed. The main hardware method is  electroencephalography  during the interictal period and, if possible, at the time of attacks. Decoding of the electroencephalogram is carried out according to the criteria of the ILAE (International Antiepileptic League).

Video monitoring is also used, which makes it possible to observe short seizures, which are very difficult to predict or stimulate.

Idiopathic epilepsy is diagnosed in cases of the absence of organic damage to the structures of the brain, for which modern methods of neuroimaging are used - computed and magnetic resonance imaging. To assess the work of the heart, electrocardiography and ECHO-cardiography are prescribed, often in dynamics and under stress. Blood pressure is monitored regularly. [18]

Also, the patient is prescribed neuropsychological, otoneurological and neuro-ophthalmological examinations, according to indications, other examinations can be prescribed.

Differential diagnosis

Differential diagnosis of idiopathic epilepsy is difficult. Firstly, in this case, structural transformations of the brain substance are not detected, secondly, the age of manifestation often does not make it possible to interview the patient, thirdly, epileptic seizures are often masked as fainting, psychogenic seizures, sleep disorders and others caused by neurological and somatic diseases.

Epileptic seizures are differentiated with a number of conditions: vegetative and psychogenic seizures, myodystonia, paroxysmal myoplegia, syncope, epileptiform seizures in acute disorders of cerebral circulation, sleep disorders, and so on. Should be alerted by the presence of a factor provoking an attack such as standing, overeating, hot baths, stuffiness; pronounced emotional component; uncharacteristic clinical picture and duration; lack of some symptoms, for example, confusion and sleep in the post-seizure period, absence of close relatives with epilepsy, and other inconsistencies. Given the severity of the disease and the toxicity of anticonvulsants, correct diagnosis often depends not only on the prognosis for recovery, but also on the patient's life. [19]

Treatment of the idiopathic epilepsy

Basically, various forms of idiopathic epilepsy to achieve long-term remission and the absence of relapses require long-term  drug therapy , especially in cases of juvenile absence and myoclonic epilepsy. In some cases, there is a lifelong need for medication. Although, for example, benign familial neonatal seizures in most cases are self-limited, therefore anticonvulsant therapy is not always considered justified, nevertheless, short-term drug treatment is sometimes prescribed. In any cases, the question of its expediency, the choice of medication and the duration of administration should be decided individually by the doctor after a thorough examination of the patient.

In idiopathic generalized epilepsy (various forms, including infantile seizures), as well as focal seizures, valproates have proved to be the most effective. With monotherapy with the drug, the effect of treatment is achieved in 75% of cases. It can be used in combination with other anticonvulsants. [20]

Preparations with the active ingredient sodium valproate (valproic acid), for example, Depakine or Konvuleks, prevent the development of typical absence seizures, as well as myoclonic, tonic-clonic, atonic seizures. Eliminate photostimulation and correct behavioral and cognitive abnormalities in patients with epilepsy. The anticonvulsant action of valproate is thought to be mediated in two ways. The main, dose-dependent, is a direct increase in the concentration of the active ingredient in the blood and, consequently, in the brain substance, it contributes to an increase in the content of γ-aminobutyric acid there, activating the inhibition processes. The second, additional mechanism of action may hypothetically be associated with the accumulation of metabolites of sodium valproate in cerebral tissues or with changes in neurotransmitters. It is possible that the drug has a direct effect on the membranes of neurons. Contraindicated in case of hypersensitivity to valproic acid derivatives, patients with chronic hepatitis, even in a family history and hepatic porphyria, with a deficiency of enzymes involved in the breakdown of auxiliary components of the drug. The development of a wide range of side effects is also dose-dependent. Adverse reactions can occur from the hematopoiesis, central nervous system, digestive and excretion organs, and the immune system. Valproic acid has teratogenic properties. Combination therapy with Lamotrigine is not recommended due to the high risk of developing allergic dermatitis up to Lyell's syndrome. The combination of valproate with herbal remedies containing the herb St. John's wort is contraindicated. Care should be taken to combine these funds with neuropsychotropic drugs, if necessary, the dose is adjusted. [21]

Clonazepam, which enhances the inhibitory effects of γ-aminobutyric acid, is effective in generalized seizures of all types. It is used for short courses of treatment and in low therapeutically effective doses. Long-term courses in idiopathic epilepsy are undesirable, the use of the drug is limited by side effects (including paradoxical ones - increased seizures and seizures), as well as a fairly rapid development of addiction. Contraindicated in patients prone to respiratory arrest during sleep, muscle weakness and confusion. Also not prescribed for sensitized individuals and patients with severe hepatic / renal failure. Possesses teratogenic properties.

Lamotrigine  controls generalized absence seizures and tonic-clonic seizures. For the control of myoclonic seizures, the drug is usually not prescribed due to the unpredictability of the action. The main anticonvulsant effect of the drug is associated with the ability to block the flow of sodium ions through the channels of the presynaptic membranes of neurons, thereby slowing down the excessive release of excitatory neurotransmitters, primarily glutamic acid, as the most common and significant in the development of epileptic seizures. Additional effects are associated with effects on calcium channels, GABA- and serotonergic mechanisms.

Lamotrigine has less significant side effects than classic anticonvulsants. Its use is allowed, if necessary, even in pregnant patients. It is called the drug of choice for generalized and focal idiopathic epilepsy.

Etosuximide is the drug of choice for simple absences (childhood absence epilepsy). But it is less effective in myoclonus and practically does not control generalized tonic-clonic seizures. Therefore, in juvenile absence epilepsy with a high risk of developing generalized tonic-clonic seizures, it is no longer prescribed. The most common side effects are limited to dyspeptic symptoms, skin rash, headache, however, sometimes there may be a change in the blood picture, tremors of the extremities. In rare cases, paradoxical effects develop - large epileptic seizures.

The new anticonvulsant Topiramate, a fructose derivative, is also recommended for the control of generalized and localized seizures of idiopathic epilepsy. Unlike Lamotrigine and classical anticonvulsants, it is not able to stop affective symptoms. The drug is still under investigation, but seizure control has been proven. Its mechanism of action is based on the blockade of voltage-dependent sodium channels, which inhibits the emergence of repeated excitation potentials. It also promotes activation of the γ-aminobutyric acid inhibition mediator. There is still no information about the occurrence of dependence when taking Topiramate. Contraindicated in children under six years of age, pregnant and lactating women, as well as persons who are hypersensitive to the components of the drug. Topiramate has a lot of side effects, like other drugs of central anticonvulsant action.

Another new drug used in the treatment of idiopathic epilepsy is Levetiracetam. Its mechanism of action is poorly understood, but the drug does not block sodium and T-calcium channels and does not enhance GABAergic transmission. It is assumed that the anticonvulsant effect is realized when the drug substance is attached to the synaptic vesicular protein SV2A. Levetiracetam also exhibits mild anxiolytic and antimanic effects.

In ongoing clinical studies, the drug has shown itself as an effective means of controlling partial seizures and as an additional drug in the complex therapy of generalized myoclonic and tonic-clonic seizures. However, research on the antiepileptic effect of Levetiracetam is still ongoing.

To date, the drugs of choice for the treatment of idiopathic generalized epilepsy with absence seizures are used in the first line of monotherapy with valproate, ethosuximide, lamotrigine, or a complex of valproate with ethosuximide. Second-line drugs for monotherapy are topiramate, clonazepam, levetiracetam. In resistant cases, polytherapy is performed. [22]

It is recommended to treat idiopathic generalized epilepsy with myoclonic seizures as follows: first line - valproate or levetiracetam; the second is topiramate or clonazepam; the third is piracetam or polytherapy.

Generalized tonic-clonic seizures are supervised by monotherapy with valproate, topiramate, lamotrigine; second-line drugs - barbiturates, clonazepam, carbomazepine; polytherapy.

In generalized idiopathic epilepsy, it is preferable to avoid prescribing classic anticonvulsants - carbamazepine, hapabentin, phenytoin and others, which can increase the frequency of seizures up to the development of an epilepticus.

Focal seizures are still recommended to be controlled with classical drugs with the active substance carbamazepine or phenytoin or valproate. In Rolandic epilepsy, monotherapy is used, anticonvulsants are prescribed in the minimum effective dosage (valproates, carbamazepines, diphenin). Complex therapy and barbiturates are not used.

In idiopathic partial epilepsies, intellectual-mnestic disorders are usually absent, therefore, experts do not consider aggressive antiepileptic polytherapy to be justified. Monotherapy is used with classical anticonvulsants.

The duration of therapy, the frequency of administration and the dose are determined individually. It is recommended to prescribe drug treatment only after a second seizure, and two years after the last one, the issue of drug withdrawal can already be considered.

In the pathogenesis of the development of seizures, there is often a deficiency of B vitamins, in particular, B1 and B6, selenium and magnesium. In patients undergoing anticonvulsant therapy, the content of vitamins and minerals, for example, biotin (B7) or vitamin E, also decreases. When taking valproates, seizure activity is reduced by levocarnitine. Vitamin D deficiency can occur, causing calcium loss and bone embrittlement. In newborns, seizures can be caused by a deficiency of folic acid, if the mother took anticonvulsants, then vitamin K deficiency can form, which affects blood clotting. Vitamins and minerals may be necessary for idiopathic epilepsy, however, the appropriateness of their intake is determined by the doctor. Uncontrolled intake can lead to undesirable consequences and aggravate the course of the disease. [23]

For current seizures, no physical therapy is needed. Physiotherapy, therapeutic exercises, massage are prescribed after six months have passed since the beginning of remission. In the early rehabilitation period (from six months to two years), different types of physical effects are used, except for all effects on the head area, hydro massage, mud therapy, skin stimulation of muscles and projections of peripheral nerves. In case of remission for more than 2 years, rehabilitation measures after the treatment of idiopathic epilepsy include the entire spectrum of physiotherapeutic procedures. In some cases, for example, if there are signs of epileptiform activity on the electroencephalogram, the question of the possibility of carrying out physiotherapeutic treatment is decided individually. The procedures are prescribed taking into account the leading pathological symptom.

Alternative treatment

Epilepsy is too serious a disease to be treated with alternative remedies these days, when seizure control drugs have appeared, at least it is unreasonable. Healer methods can be used, but only after they are approved by the attending physician. Unfortunately, they cannot replace carefully selected medicines, and in addition, they can reduce their effectiveness.

Perhaps it would be quite safe to take a bath with a decoction of hay from grass grown in the forest. This is how epileptics were treated in the old days.

Another popular method that can be tried in the summer, for the townspeople, for example, in the country. It is recommended to go outside in the early summer morning, before the morning dew has dried up, and spread on the grass a large towel, sheet, bedspread made of natural fabric - cotton or linen. It should be saturated with dew. Then wrap the patient with a cloth, lay or sit down, do not remove until it dries on his body (the method is fraught with hypothermia and colds).

The aroma of the resin of the myrrh tree (myrrh) has a very beneficial effect on the nervous system. It was believed that a patient with epilepsy should inhale the scent of myrrh around the clock for a month. To do this, you can fill an aroma lamp with myrrh oil (a few drops) or bring pieces of resin from the church and disperse the suspension from them in the patient's room. Just keep in mind that any smell can cause an allergic reaction.

The use of freshly squeezed juices will compensate for the lack of vitamins and minerals during the period of taking medicinal anticonvulsants.

Recommended juice from fresh cherries, a third of a glass twice a day. This drink has an anti-inflammatory and bactericidal effect, soothes, relieves vascular spasms, relieves pain. Capable of binding free radicals. Improves blood composition, prevents the development of anemia, removes toxins. Cherry juice is one of the healthiest, it contains B vitamins, including folic and niacin, vitamins A and E, ascorbic acid, iron, magnesium, potassium, calcium, sugar, pectins and many other valuable substances.

Also, as a fortifying agent, you can take juice from green sprouts of oats and its spikelets in the stage of milk ripeness. This juice, like others, is drunk before meals, a third of a glass, two or three times a day. Young sprouts of oats have a very valuable composition: vitamins A, B, C, E, ferments iron, magnesium. The juice will cleanse the blood and restore its composition, raise immunity, and normalize metabolism.

It is also possible to prepare decoctions, infusions, teas from medicinal plants and use them to strengthen the immune system, the nervous system and the body as a whole. Herbal treatments cannot replace anticonvulsants, but can complement their effects. Plants with calming properties are used - peony, motherwort, valerian. St. John's wort, according to alternative healers, can reduce the frequency of seizures and reduce anxiety. It is a natural anxiolytic, however, it is not compatible with valproate.

An infusion of mountain arnica flowers is taken in a single dose of 2-3 tablespoons before meals three to five times a day. Insist a tablespoon of dried flowers, filled with a glass of boiling water, for an hour or two. Then filter.

Angelica rhizomes are dried, crushed and taken in the form of an infusion, half a glass before meals three to four times a day. The daily rate is brewed as follows: 400 ml of boiling water is poured over two tablespoons of vegetable raw materials. After two to three hours, the infusion is filtered and drunk warm, each time slightly warming up before taking.

Homeopathy

Homeopathic treatment of idiopathic epilepsy should be supervised by a homeopathic physician. There are enough funds for the treatment of this disease: Belladonna

Belladonna (Belladonna) is used for atonic seizures, convulsions, and the drug can also be effective for partial epilepsy with auditory symptoms.

Bufo rana relieves nocturnal seizures well, it does not matter whether the patient wakes up or not, and Cocculus indicus - seizures that occur in the morning when the patient wakes up.
Mercury (Mercurius), Laurocerasus (Laurocerasus) is used for seizures with an atonic component and tonic-clonic seizures.

There are many other drugs used to treat epileptic syndromes. When prescribing homeopathic remedies, not only the leading symptoms of the disease are taken into account, but also the constitutional type of the patient, his habits, character traits and preferences.

In addition, homeopathy can help you recover quickly and efficiently after a course of treatment with anticonvulsants.

Surgery

Surgery is a radical treatment for epilepsy. It is performed with an immunity to drug therapy, frequent and severe focal seizures, causing irreparable harm to the health of patients and significantly complicating their life in society. In idiopathic epilepsy, surgery is rare because it responds well to conservative therapy.

Surgical operations are highly effective. Sometimes surgical treatment is carried out in early childhood and avoids cognitive impairment.

Preoperative examination is of great importance to establish true pharmacoresistance. Then, the location of the epileptogenic focus and the volume of surgery are determined as accurately as possible. Epileptogenic areas of the cerebral cortex are removed or disabled using multiple incisions in focal epilepsy. For generalized epilepsy, hemispherotomy is recommended - a surgical procedure that results in the termination of pathological impulses that cause seizures between the cerebral hemispheres. 

A stimulator is also implanted in the clavicle region, which acts on the vagus nerve and helps to reduce the pathological activity of the brain and the frequency of seizures. [24]

Prevention

It is almost impossible to prevent the development of idiopathic epilepsy, however, even women with epilepsy have a 97% chance of having a healthy baby. It is enhanced by a healthy lifestyle of both parents, a safely borne pregnancy, natural childbirth.

Forecast

The vast majority of cases of idiopathic epilepsy are benign and have a good prognosis. Complete therapeutic remission is achieved on average in more than 80% of patients, although some forms of the disease, especially those developing in adolescents, require long-term antiepileptic therapy. Sometimes it is lifelong. [25] However, modern drugs are mainly used to control seizures and provide patients with a normal quality of life.