Epilepsy: an overview of information

Epilepsy is one of the most common and serious neurological diseases that occurs at any age. Despite significant advances in the diagnosis and treatment of this disease, in many patients the existing methods of treatment do not allow to achieve adequate control of seizures or cause significant side effects.

Epileptic seizure is a pathological, uncontrolled outbreak of electrical activity in neurons of the gray matter of the cerebral cortex, temporarily disrupting the normal function of the brain. It is usually accompanied by a brief episode of altered consciousness with motor, sensory and behavioral disorders.

[1], [2], [3], [4], [5], [6],

Causes of the epilepsy

Isolated epileptic paroxysm can be provoked in healthy people by reversible stressors (for example, hypoxia, hypoglycemia, children with fever). The diagnosis is made when the patient has two or more cases of epilepsy, not associated with the action of reversible stressors.

The etiology is symptomatic (if there is a known cause, such as a brain tumor or stroke) or idiopathic (the cause is not known) epilepsy. Idiopathic, perhaps, has a genetic basis.

In generalized seizures, aberrant electrical activity diffusively involves the entire cortex of both hemispheres from the very beginning, with a loss of consciousness usually observed. Generalized crises are more often associated with impaired metabolism in the brain, including those caused by genetic factors. Generalized also include neonatal seizures and absences, tonic-clonic, atonic and myoclonic paroxysms.

Partial (focal) seizures often develop due to focal structural disorders. Pathological neuronal activity begins in one section of the cortex. Partial crises can be simple (without disturbance of consciousness) or complex (with a change in consciousness, but without losing it completely). Sometimes, in focal lesions, the excitement emanating from it so quickly covers both hemispheres of the brain, which immediately generates a crisis, when focal manifestations do not yet have time to develop, or generalized paroxysm follows a short focal (called secondary generalization).

Etiological factors

Condition	Examples
Autoimmune diseases	Cerebral vasculitis, multiple sclerosis (rarely)
Edema of the brain	Eclampsia, hypertensive encephalopathy, ventricular obstruction
Cerebral ischemia	Adams-Stokes Syndrome, cerebral venous thrombosis, embolic cerebral infarction, vasculitis
Brain Injury	Birth trauma, skull fracture, penetrating injuries
Infections of the central nervous system	HIV, cerebral abscess, 4-day malaria, meningitis, neurocysticercosis, neurosyphilis, toxoplasmosis, viral encephalitis
Congenital anomalies	Genetic disorders (eg, fits of the fifth day, lipidosis, for example, Tay-Sachs disease), diseases associated with violation of migration of neurons (ie heterotopia)
Medications	Causes paroxysms: cocaine, other stimulants of the central nervous system, cyclosporine, tacrolimus, pentylenetetrazole, picrotoxin, strychnine Decrease the threshold of epileptic activity: aminophylline, antidepressants, sedative antihistamines, antimalarials, some antipsychotics (eg clozapine), buspirone, fluoroquinolone, theophylline
Extensive brain damage	Intracranial hemorrhages, tumors
Hyperthermia	Fever, heat stroke
Metabolic disorders	Usually hypoglycemia, hyponatremia; less often aminoaciduria, hyperglycemia, hypomagnesemia, hypernatremia
Pressure change	Decompression disease, hyperbaric oxygenation
Cancellation Syndromes	Alcohol, anesthetics, barbiturates, benzodiazepines

Fits of the fifth day (benign neonatal) are tonic-clonic crises developing between the 4th and 6th days of life in healthy newborns; one form is hereditary.

Idiopathic epilepsy usually begins between the ages of 2 and 14 years. The frequency of symptomatic paroxysms is highest in newborns and elderly people. At the age of 2 years, they are usually the result of developmental defects, birth trauma or metabolic disorders. A significant proportion of them, appearing in adulthood, are secondary and are caused by brain trauma, alcohol withdrawal, tumors or cerebrovascular diseases; in 50% of cases the etiology of crises remains unknown. Cases of epilepsy of the elderly are most often due to a brain tumor or stroke. Post-traumatic seizures after craniocerebral trauma with skull fractures, intracranial hemorrhages or focal neurologic defect develop in 25-75% of cases.

Cases of simulating epileptic paroxysms by persons with psychiatric disorders are defined as non-epileptic, or pseudo-seizures.

[7], [8], [9], [10],

Pathogenesis

An epileptic fit occurs as a result of a disturbance in the balance between the excitatory and inhibitory systems in the brain. Different types of disease are mediated by different physiological mechanisms and are associated with the defeat of various zones of the brain. Some antiepileptic drugs increase inhibitory effects in the central nervous system, facilitating GABA-ergic transmission, while others weaken the exciting afferentation, reducing the activity of glutamatergic systems. Some antiepileptic drugs block fast discharges of neurons, interacting with the sodium channels of nerve cells. Since the appearance in 1912 of phenobarbital several dozens of antiepileptic drugs have been developed. To date, there is not one single drug that would be more effective than others, since none of them is effective for all types of crises in all situations. In this regard, the choice of medication is based on an accurate diagnosis and clinical response.

Many of the problems associated with this disease are not only medical, but also psychosocial. In cases where seizures are not controlled by drugs, other methods of treatment may be effective, for example, neurosurgical intervention. The ultimate goal of any method of treating epilepsy is to eliminate the cases of this pathology and improve the quality of life of patients.

[11], [12], [13], [14], [15], [16],

Symptoms of the epilepsy

An epileptic fit can be preceded by a sensory aura or mental manifestations (for example, the smell of rotting flesh, the phenomenon of a fluttering butterfly in the abdomen). Most of them end spontaneously within 1-2 minutes. Immediately after the convulsions (usually generalized), there comes an after-illness, the patient falls into a deep sleep, and when he wakes up, does not remember anything, complains of general weakness, weakness, headaches. Sometimes Todd's paralysis develops (transient paralysis of the part of the body involved in the attack). Post-admission condition usually lasts from a few minutes to an hour.

Between seizures, those suffering from this pathology, as a rule, look neurologically healthy people, although large doses of anticonvulsants depress psychomotor reactions. Any aggravation of mental or psychiatric disorders is usually due to the underlying neurological disorder that caused the disease to develop, rather than crises per se. In rare cases, the disease is refractory to therapy (status epilepticus).

Simple partial (focal, focal) seizures

Simple partial seizures begin with certain motor, sensory or psychomotor focal manifestations and are not accompanied by loss of consciousness. Specific symptoms indicate the affected area of the brain. With Jacksonian paroxysms, focal motor manifestations begin in the hand or foot and then spread throughout the limbs. Some focal crises begin with the face, then the cramps cover the arm and sometimes the leg. Some focal motions are manifested by raising their hands and turning their heads toward the moving arm. Sometimes they become generalized.

[17], [18]

Complex (complex) partial seizures

A complex partial attack is often preceded by an aura. During the case of epilepsy, the patient for a short time loses contact with the environment, the eyes are wide open, the sight is directed to one point; he can make automatic aimless movements or make inarticulate sounds. He does not understand the speech addressed to him and sometimes resists attempts to help him. Epilepsy lasts 1-2 minutes, the state of confusion is still 1-2 min after the seizure, but a relative understanding of what is happening (purposefully avoid painful stimuli). The patient can attack a person trying to keep him during a paroxysm, but unprovoked aggressive behavior is uncharacteristic.

When the focus is located in the left temporal lobe, paroxysms can cause violation of verbal memory, with localization in the right temporal lobe - a disorder of spatial visual memory. In the interictal period, patients with a temporal form of the disease are more likely to have psychiatric disorders than in the whole population: serious psychological problems are detected in 33% of patients, symptoms of schizophreniform or depressive psychosis - in 10%. Characteristic changes in behavior, in particular the appearance of excessive religiosity, or a pronounced dependence on other people, or a tendency to hypergraphy (a style of writing characterized by excessive verbosity, pedantic insistence in mentioning many unimportant details and a tendency to obsessive insertions) or a change in sexual behavior.

[19], [20]

Epilepsia partialis continue

This is a rare form of central focal motor paroxysms, usually involving the arm or half of the face; Paroxysms following one after another at intervals of several seconds or minutes appear as periods lasting several days, weeks, and sometimes even years. The cause of epilepsia partialis continue in adults is usually a structural lesion of brain tissue. In children, this is usually a focal inflammatory process of the cerebral cortex (eg, Rasmussen's encephalitis) due to a chronic viral infection or an autoimmune disease.

[21], [22]

Generalized seizures

They flow with loss of consciousness and motor impairments from the very beginning of the attack.

For infantile spasms (salamative convulsions) is characterized by sudden bending of the hands with the torso bending forward and extending the legs. Attacks last only a few seconds, but can be repeated many times during the day. They happen only in the first 5 years of life, and later they can be replaced by other types of crises. Usually there are signs of organic brain damage.

Absenses (formerly petit mal) are manifested by loss of consciousness for 10-30 seconds, with the loss or preservation of muscle tone. The patient does not fall, there is no seizure, but at the same time he suddenly stops any activity and resumes it at the end of the crisis. The post-adherence period itself, as well as the awareness of what happened, does not happen. Absenses are genetically determined and arise, mainly, in children. Without treatment, absences are repeated many times a day, mostly in a calm environment. Paroxysms can be triggered by hyperventilation, but rarely - during physical exertion. Atypical absences last longer, are accompanied by more obvious twitching or automatic movements and are accompanied by a less pronounced loss of awareness of what is happening. Most patients have a history of organic brain damage, developmental delay, and other types of seizures. Atypical absences usually continue in adulthood.

Atonic seizures occur in children

They are characterized by a short-term complete loss of muscle tone and consciousness, which leads to falls and significantly increases the risk of injury, especially craniocerebral.

Generalized tonic-clonic paroxysms (primary generalized) usually begin with an involuntary scream, followed by loss of consciousness and a fall with tonic and then clonic cramps of the limbs, trunk and head. Sometimes during an attack there are involuntary urination and defecation, foam from the mouth. Epilepsy usually lasts 1-2 minutes. Secondary-generalized tonic-clonic paroxysms begin with simple or complex partial crises.

Myoclonic cases of epilepsy are short, lightning convulsions of one or more extremities, or torso. They can repeat themselves many times, turning into a tonic-clonic crisis. Unlike other seizures with bilateral motor disorders, consciousness is not lost unless generalized paroxysm develops.

Juvenile myoclonic epilepsy develops in childhood or puberty. Bilateral myoclonic crises consist of single or short arrhythmic jerks of the hands, sometimes of the lower extremities, usually with a clear consciousness, which in 90% of cases go to generalized tonic-clonic convulsions. Seizures are often provoked by lack of sleep, alcohol consumption and are often in the morning during awakening.

Febrile convulsions occur with an increase in body temperature, but there should be no signs of intracranial infection. Febrile convulsions occur in about 4% of children between the ages of 3 months to 5 years. Benign febrile convulsions are short-lived, single and generalized tonic-clonic. Complicated febrile seizures are focal, last more than 15 minutes and are repeated two or more times throughout the day. In patients with febrile convulsions, the probability of occurrence of repeated afebrile seizures in the future is increased, in 2% the disease develops. The likelihood of the development and recurrence of cases of the disease is further increased in children with complicated febrile seizures, with a previous neurological pathology, with the onset of paroxysms before the age of 1 year, or the presence of epilepsy in a family history.

[23]

Epileptic status

With epileptic status, generalized tonic-clonic convulsions (two or more crises) follow each other for 5-10 minutes, and in the intervals between them the patient does not regain consciousness. Previously adopted for the definition of this nosology, the time interval "more than 30 minutes" was revised in order to promptly provide medical care. In the absence of care, a generalized seizure lasting more than 1 hour leads to permanent damage to the brain and can result in a fatal outcome. Among the many reasons that provoke its development, the most frequent is the withdrawal of anticonvulsants. In complex partial crises or absences, it often manifests itself as a prolonged disturbance of consciousness.

[24], [25], [26]

Behavior

Epilepsy is of interest to forensic psychiatrists because of its effect on consciousness (which may be related to the commission of a crime) and the possible etiological association with behavioral disorder (including crime) between seizures.

[27], [28], [29], [30], [31], [32]

Aura

It is the primary focus of the attack and comes before losing consciousness. The subject is aware of the presence of various experiences, which are determined by the zone of discharges in the brain, and are able to recall them later. Usually with an aura unintended limb movements, discrete sensations, emotions, various hallucinations and intrusive thoughts are noted. Aura can go into full paroxysm, but it may not go.

[33], [34], [35], [36], [37],

Complete loss of consciousness

It can be very brief, as, for example, with petit mal, or last a few minutes, as, for example, with grand mal. The state of stupor is also described, which can occur with petit mal as a result of rapidly following one after another cases of the disease.

[38], [39], [40]

Epileptic automatism

With an abnormal picture of the electrical activity of the brain, usually in the temporal region (different complex partial seizures), a person may have a difficult and partially purposeful activity. This activity is carried out in a state of darkened consciousness, although at the same time a person is able to control the position of his body and muscle tone. Automatism usually lasts from a few seconds to several minutes, mostly less than five minutes, although in rare cases it can last longer (psychomotor status). Such a subject seems to the external observer to be somewhat dumbfounded, or his behavior seems inadequate in this situation. The culmination can be grand mal. Such a subject usually has a memory of automaticity. Theoretically, in this state, a "crime" can be committed if, for example, the subject had a knife in the beginning of automatism, and then he continued to make cutting movements.

[41], [42], [43], [44], [45]

Fugues

This behavioral disorder resembles a complex epileptic automatism, but lasts much longer (several hours or days). During this time, trips can be made somewhere, purchases can be made, etc. In any case, this behavior looks somewhat strange. The subject does not retain such an event in memory. Great problems can be caused by the problem of differentiation of epileptic and psychogenic fugues, which really coincide in many respects. This can be helped by the presence in history of seizures, abnormal EEG and the presence of a fugue in the anamnesis.

Twilight states

Lishman recommends limiting the use of this term to episodes of time-consuming abnormal subjective experiences, lasting up to several hours with a disturbed consciousness. This is a dreamlike scattered behavior and a slow reaction. The degree of reaction to the environment can be very different. The subject experiences a strong sense of panic, horror, anger, or rapture; he can sit quietly during an attack, but he may have sudden outbreaks of aggressive or destructive behavior. Such subjects can be very irritable and can give outbursts of rage to any attempt at intervention. This can lead to the commission of a "crime". These experiences are accompanied by impaired electrical activity of the brain, often with localization of the focus in the temporal region. This state can be completed by grand mal.

Postictal conditions

After ictus a person may have difficulty recovering full consciousness. Such a subject looks confused and awkward. He is irritated, and there may be aggressive behavior (which can lead to crime), which is usually a reaction to undesirable interference by others. Sometimes there is a postictal twilight state, which can last from several hours to several days and is characterized by inhibition, hallucinations and affective disorder, or postictal paranoid psychosis.

Behavioral Disorders Between Attacks

The relationship between epilepsy and frustrated behavior in the period between seizures should be designated as complex. This may be due to changes in the brain that caused the disease, or changes in the brain due to severe form or due to medication; this can also be the result of the psychological impact of suffering pathology. As a possible cause of the disturbed behavior between attacks, any associated mental disorders or mental illnesses are also mentioned.

As a result of the above factors, the subject can experience:

• changes in the emotional state or personality;
• conditions similar to mental illness;
• some degree of mental retardation; or
• violations of sexual behavior.

[46], [47], [48], [49]

Changes in the emotional state, behavior or personality

[50],

Seizure of seizures

Some subjects (most often with the temporal form of the disease) notice changes in their emotional state in a few hours or days before the grand mal. Usually this is an unpleasant condition with increased irritability, stress and a gloomy mood. This emotional state can be associated with difficult behavior. In such a state, it is not impossible to commit an attack on another person.

Behavioral disorder in children

It is shown that in children suffering from certain types (especially temporal epilepsy), the probability of manifestations of antisocial behavior is higher than normal. This behavior is not directly related to seizures and is quite likely a consequence of the complex interaction of many factors, including brain damage, negative family influences, such as paroxism, the child's psychological reaction to the disease, the effect of drug therapy and the effect of hospitalization or placement in a specialized institution. Children with petit mal are less aggressive than children with grand mal.

[51], [52], [53], [54], [55]

Personality disorders in persons

It is now generally accepted that there is no separate epileptic personality disorder. Those features of behavior that were previously considered to be the result of epileptic personality disorder are now understood as the result of a joint manifestation of brain damage factors, the institutionalization and effects of anticonvulsants of the old generation. Such personality traits as aggression are more common in the temporal form of the disease. In that small part of persons who have a personality disorder, its etiology is likely to be multifactorial. These factors include psychosocial effects, effects associated with brain damage, abnormal electrical activity between crises and the effect of anticonvulsants.

Mental limitation

Epilepsy is much more common among people with mental retardation. This is a reflection of a deep brain disorder that can underlie both conditions. Obviously, severe seizures can lead to brain damage, which can exacerbate an already existing degree of a person's mental limitations. Among those with a severe degree of mental retardation, 50% had an epileptic seizure in history. However, if you exclude the defeat of the brain, then the intelligence of children is within normal limits.

Sexual dysfunction

A number of studies have consistently described a decrease in libido and impotence in individuals. However, if we exclude the assumption of lowering the levels of male sex hormones, the direct link between the disease and sexual dysfunction is not accepted by specialists. Hyperexuality is rare. In some rare cases, a connection with temporal epilepsy of fetishism and transvestism is shown. In the literature, cases have been described that claimed that surgical removal of the focus in the temporal region cured fetishism. However, it is not clear whether there was really a direct link with the temporal form of the disease or sexual dysfunction was the result of distorted human relationships due to the subject.

Crimes

In the XIX century, epilepsy or a tendency to it was considered a feature of many criminals. And according to the concepts of the time, crimes committed in blind rage were also considered a manifestation of the epileptic process. Modern research refutes this view. Studies of patients attending outpatient clinics did not find them to be excessively criminal. However, a more complete study of Gudmundsson all Icelanders, revealed a slight overstatement of crime in men with this pathology. Gunn showed that the prevalence of pathology in English prisons is higher than in the general population: among prisoners, 7-8 people per thousand suffered from the disease, while in the general population 4-5 people. In a study of 158 prisoners, there was no convincing evidence of the commission of a crime in a state of automaticity, although ten people committed crimes immediately before or immediately after the onset of the attack. In a study of 32 people in special hospitals, two at the time of the crime could be in a state of post-confusion. That is, although epilepsy may indeed be one of the factors leading to antisocial behavior in some cases, in general this relationship is not expressed among individuals, and crimes during a crisis are rarely committed.

1. Crime can occur in a disturbed state, the cause of which is directly paroxysm. This happens rarely.
2. Crime and attack can be an accidental coincidence.
3. The defeat of the brain due to epilepsy could cause personal problems, which resulted in antisocial behavior.
4. A subject can develop a strong antisocial attitude to phenomena as a result of the difficulties experienced by him in life because of illness.
5. A deprivation environment in early childhood can both generate an antisocial attitude to phenomena, and subject the subject to the effects of epileptogenic factors.
6. Antisocial subjects are more likely to get into dangerous situations and suffer more head injuries that can cause illness than normal.

[56], [57], [58], [59],

EEG changes and violent crimes

Violence, most likely, can not be attributed to common phenomena in direct connection with an epileptic seizure. Usually any violence in connection with a seizure happens in a state of post-confusion and consists in an attack on people who somehow interfere with the situation. Violence can also happen (very rarely) with epileptic automatism. It was also described in connection with discharges in the amygdala. Most of the violence committed by individuals occurs during periods between crises. Surveys of scientific studies on the subject of increased prevalence of violence in persons with the disease, give different results. Thus, in the study of 31 subjects with temporal epilepsy directed to the clinic, 14 had aggression in history. Violence was usually easy and did not correlate with EEG or scan (CAT). However, this behavior correlated with the male sex, the presence of behavioral disorders from childhood (which often led to training in special residential institutions), personal problems in adulthood and dim intelligence. And, of course, violence can occur in the case of psychosis.

It was also assumed that EEG changes are more often observed in persons who committed violent crimes. This view is based on the results of a classic study that established that EEG abnormalities were more pronounced if the murder was impulsive or unmotivated. Wiliam argued that impulsive men with violent manifestations had elevated levels of anomalies in the temporal region. Together, those data were not confirmed by other researchers, and therefore they are treated with considerable caution. Gunn and Bonn, for example, found no connection between temporal epilepsy and violence. Lishman's study, performed on faces that had suffered head trauma, confirmed that the most common lesions with the aggression were lesions of the frontal brain regions. Driver et al. It was not possible to detect significant differences between the EEG of killers and EEG of persons without characteristics of violence, if the EEG researcher had no information about these individuals in advance.

[60], [61], [62], [63]

Evaluation of the offender

Fenwick proposed the following six criteria in order to help psychiatrists determine the degree of veracity of the subject's statement that the crime committed by him occurred in a state of disturbed consciousness.

1. About the patient should be aware that he suffers from this pathology, that is, it should not be his first attack.
2. The perfect action must be uncharacteristic for this person and inadequate circumstances.
3. There should be no signs of intent, as well as attempts to conceal the crime.
4. Witnesses of crime must describe the state of the offending state of the offender, including the description of the subject when he suddenly realizes what is happening, and his confusion at the moment of stopping automatism.
5. There must be amnesia of the entire period of automatism.
6. There should be no memory disturbances that precede automatism.

The diagnosis of epilepsy and epileptic automatism is a clinical diagnosis. Special studies such as magnetic resonance spectroscopy, computed tomography and EEG may be useful, but they are not able to prove or exclude the presence of automatism.

[64], [65]

Forms

Epilepsy is classified in ICD-10 as a disease of the nervous system, not a mental disorder, but it acquires significance in connection with the effects it produces on the mental state of the subject. It is divided into generalized and focal (or partial).

The generalized in turn is divided into the primary generalized with two different forms - grand mal and petit mal, and secondary generalized, which is noted when focal epilepsy seizes the thalamo-cortical pathways, thus causing generalized paroxysm. This can lead to grand mal with a prior aura.

Grand mal is characterized by a tonic phase, followed by a clonic phase and a period of unconsciousness lasting several minutes. With petit mal, only moments of loss of consciousness occur, and the patient immediately resumes his normal activity. Absence is manifested for the external observer in the transitory "empty" expression of the face and, possibly, in the light twitching of limbs or eyelids, akinetic fit, expressed in a sudden fall, and myoclonic, sharp movement of the elongated limb.

In focal (partial) epilepsy, such attacks begin in the cortical part of the brain. The symptoms accordingly depend on the involved area of the brain. If only a part of the brain is involved, then there may be a conscious sensation (aura). The nature of the sensation gives the key to determining the discharge zone. Focal form in turn is divided into simple partial (focal) paroxysms without damage to consciousness and complex partial focal seizures with complex movements and impaired consciousness (mainly occurring in the temporal region).

[66], [67], [68], [69]

Diagnostics of the epilepsy

First of all, you should make sure that the patient has epileptic paroxysm, and not fainting, an episode of cardiac arrhythmia or symptoms of drug overdose, then identify possible causes or provoking factors. At the onset of the disease, an examination is shown in the intensive care unit, with an earlier diagnosis in an outpatient setting.

[70], [71], [72], [73]

Anamnesis

The presence of an aura, classical epileptic seizures, as well as biting the tongue, involuntary urination, prolonged loss of consciousness and a state of confusion after a crisis testify to an epileptic attack. When collecting anamnesis, you should get information about the first and subsequent crises (duration, frequency, sequence of development, the longest and shortest interval between seizures, the presence of aura and post-admission condition, provoking factors). It is necessary to identify the potential causes of symptomatic epilepsy (previous cerebral trauma or CNS infection, existing neurological disorders, drug use or withdrawal, violation of the anticonvulsant regimen, the presence of seizures or neurologic disorders in the family history).

[74], [75],

Physical examination

The data of the physical examination are almost always normal with idiopathic forms, but with serious symptoms, serious abnormalities can be detected. In the presence of fever and rigidity of the neck muscles, meningitis, subarachnoid hemorrhage or encephalitis should be excluded. Stagnant optic discs indicate an increase in intracranial pressure. Focal neurological disorders (eg, asymmetry of reflexes or muscle strength) are a sign of structural damage to the brain (eg, a tumor). Skin lesions occur in neurocortical diseases (in particular, spots in the axillary region or spots of the color "coffee with milk" with neurofibromatosis, hypopigmentation spots on the skin or shagreen plaques in tuberous sclerosis).

Study

Patients with an established diagnosis and no deviations from the neurological examination show only the determination of the anticonvulsant concentration in the blood, provided that no signs of craniocerebral trauma or metabolic disturbances were found.

If it is a first-ever attack or if the patient has a pathology in a neurologic examination, an emergency CT scan of the brain is shown to exclude focal lesions or hemorrhage. If no changes were detected on CT, MRI is performed because of better resolving power in detecting tumors and brain abscesses, cerebral venous thrombosis and herpetic encephalitis. Patients with metabolic disorders are prescribed extended laboratory tests, including clinical and biochemical blood tests (glucose, urea nitrogen, creatinine, Na, Ca, Mg and P and hepatic enzymes). If there is a suspicion of meningitis or CNS infection, CT of the brain and lumbar puncture are performed in the absence of abnormalities in CT. EEG allows to diagnose the status epilepticus with complex partial paroxysms and absences.

In patients with complex temporal partial paroxysms, changes in the EEG are also observed in the interictal (interictal) period in the form of spike waves or slow waves. In generalized tonic-clonic crises in the interictal period, symmetrical flares of acute and slow activity with a frequency of 4-7 Hz are recorded on the EEG. With secondary generalized seizures on the EEG, pathological focal activity is determined. For absences, spike waves with a frequency of 3 / s are characteristic. With juvenile myoclonic epilepsy, multiple spike waves with a frequency of 4-6 Hz and pathological waves are recorded.

Nevertheless, the diagnosis is made on the basis of the clinical picture and can not be excluded with normal EEG. With rare attacks, the probability of EEG confirmation of epilepsy is reduced. In patients with a confirmed diagnosis in 30% of cases, the first EEG has no pathological changes; The second EEG, performed after sleep deprivation, reveals pathology in only 50% of cases. In some patients, there is never a pathological change on the EEG.

Video monitoring of EEG for 1-5 days is used to identify the type and frequency of seizures (differentiation of the frontal from a fake) and to monitor the effectiveness of treatment.

Treatment of the epilepsy

The optimal approach is aimed at eliminating possible causes of symptomatic paroxysms. If a potential cause can not be established, the use of anticonvulsants, usually after the second case of epilepsy, is indicated. The expediency of prescribing anticonvulsant therapy after one (sometimes single) crisis is debatable, potential risks and benefits should be discussed with the patient.

During the seizure, the main task is to prevent injury. It is necessary to release the neck from the restraining clothes and put a pillow under the head to prevent aspiration. Do not try to prevent damage to the tongue, as there is a great risk of damaging the patient's teeth or the fingers of the caregiver. These activities should be known to family members and work colleagues.

Prior to achieving adequate control of cases of the disease, one should refrain from certain activities in which loss of consciousness can be life threatening (driving, swimming, climbing a mountain, bathing in a bath). When complete control is achieved (usually more than 6 months), these types of activity are resolved by observing precautionary measures (for example, in someone's presence). A healthy lifestyle with moderate physical activity and participation in social activities is encouraged. Some cases are subject to notification (for example, to transport control, according to local legislation), although in the absence of a case of pathology within 6-12 months the patient may be allowed to drive the vehicle.

It is recommended to avoid drinking alcohol and drugs, because cocaine, phencyclidine and amphetamines can trigger crises. It is also desirable to exclude all drugs that lower the threshold of seizure activity (in particular, haloperidol, phenothiazine).

Family members need to develop a reasonable line of behavior in relation to the patient. Excessive care, which generates a sense of inferiority, is better replaced by support and empathy, allowing to overcome these and other psychological problems, which prevents additional disability of the patient. Inpatient psychiatric care is indicated only in cases of serious mental disorders or with frequent severe attacks that do not lend themselves to pharmacotherapy.

First aid

Most cases of pathology are resolved on their own within a few minutes and do not require emergency pharmacotherapy.

Emergency intervention is required to arrest epileptic status and crises lasting more than 5 minutes, while controlling the parameters of breathing. In the presence of signs of obstruction of the airways, the patient is intubated and, having provided intravenous access, begins the injection of lorazepam in a dose of 0.05-0.1 mg / kg at a rate of 2 mg / min. If necessary, increase the dose. If the case of epilepsy could not be stopped after the administration of 8 mg of lorazepam, additionally administering phosphenytoin at a dose of 10-20 EF (phenytoin equivalents) / kg intravenously at a rate of 100-150 EF / min; second-line drug is phenytoin - in a dose of 15-20 mg / kg intravenously at a rate of 50 mg / min. In case of repeated attacks, 5-10 EF / kg of phosphenytoin or 5-10 mg / kg of phenytoin are additionally administered. Persistence of seizures after the administration of lorazepam and phenytoin indicates a refractory epileptic status when third-line drugs - phenobarbital, propofol, midazolam or valproate are required. Phenobarbital is administered at a dose of 15-20 mg / kg intravenously at a rate of 100 mg / min (children 3 mg / kg / min); if the cases of pathology continue, you should add phenobarbital at the rate of 5-10 mg / kg or valproate at a dose of 10-15 mg / kg intravenously. If the status epilepticus can not be quenched after the listed measures, the patient is intubated under general anesthesia. It is difficult to recommend an optimal anesthetic, but in most cases propofol is preferred - at 15-20 mg / kg at a rate of 100 mg / min or phenobarbital at 5-8 mg / kg (initial dose) followed by infusion 2-4 mg / kg / h before suppressing signs of activity on the EEG. Inhalational anesthetics are rarely used. After arresting the epileptic status, first and foremost identify and eliminate its cause.

The appointment of anticonvulsants for preventive purposes is appropriate for patients who have suffered a craniocerebral trauma with fractures of the skull bones, intracranial hemorrhage or focal neurologic symptoms. The use of anticonvulsant therapy reduces the risk of a pathological event within the first week after the injury, but does not prevent post-traumatic epilepsy after several months or years. If there was no seizure in an acute period of the injury, anticonvulsant therapy is canceled after 1 week.

[76],

Prolonged medication

There is no universal remedy effective for all types of crises, and different patients need different medications, and sometimes one drug is not enough.

With newly diagnosed epilepsy, monotherapy is prescribed, for which one of the anticonvulsant drugs is selected taking into account the type of seizures. Initially, the drug is prescribed in relatively low doses, then the dose is gradually increased within 1-2 weeks to the standard therapeutic level (based on the patient's body weight) while evaluating the tolerance to the drug. After about a week of treatment, the standard dose is determined by the concentration of the drug in the blood. If at the subtherapeutic level the patient continues seizures, the daily dose is gradually increased. If the patient has symptoms of intoxication, and cases of pathology continue, the dose is reduced and the second drug is gradually added. In the treatment with two agents, special care is needed, since their interaction may increase the toxic effect due to a slowing of their metabolic degradation. Then the dose of the inefficient drug is gradually reduced to a complete cancellation. If possible, it is advisable to avoid polypharmacy and not to prescribe several anticonvulsants at the same time in connection with an increase in the frequency of side effects and inter-drug interactions; The appointment of a second drug helps about 10% of patients, with the frequency of side effects more than doubling. Additional drugs can increase or decrease the concentration of the main anticonvulsant, therefore, when choosing a therapy, the doctor should first of all consider the aspects of inter-drug interaction.

As soon as the cases of the disease have completely stopped under the action of the selected medicine, it is necessary to continue taking it without interruption for 1-2 years, after which the drug can be tried to cancel, reducing the dose by 10% every 2 weeks. Approximately half of the patients in the future no crises occur without treatment. Relapse is most likely if cases of epilepsy were noted since childhood if more than one drug was required to control paroxysms if the seizures continued against the background of anticonvulsant therapy, if the cases are partial or myoclonic, and if the patient has encephalopathy or pathological changes on the EEG in during the previous year. In these cases, relapse within the first year after the cancellation of anticonvulsant therapy occurs in 60%, and in the second - in 80% of patients. If seizures from the very beginning were poorly controlled by drugs, resumed when trying to cancel treatment or are extremely undesirable for the patient due to social reasons, then treatment should be carried out indefinitely.

After the reaction to the drug is established, the data on its content in the blood are much less important for the doctor than the clinical course. In some patients, the toxic effect is manifested even with a low content of the drug in the blood, others tolerate high doses well, so monitoring the concentration serves only as an auxiliary guideline for the doctor. An adequate dose of any anticonvulsant is the lowest dose, completely stopping seizures with minimal side effects, regardless of the value of its concentration in the blood.

In generalized tonic-clonic crises, the drugs of choice are phenytoin, carbamazepine, and valproic acid (valproate). The adult daily dose of phenytoin is divided into several doses or the entire dose is prescribed for the night. If the cases of epilepsy do not stop, the dose can be gradually increased to 600 mg / day under the control of the content of the drug in the blood. At a higher daily dose, dividing it into several doses makes it possible to weaken the toxic effects of the drug.

In complex partial (psychomotor) seizures, the drug of choice is carbamazepine and its derivatives (eg, oxcarbazepine) or phenytoin. Valproates are less effective, and the clinical advantage of new quite effective drugs - gabapentin, lamotrigine, tiagabine, topiramate, vigabatrin and zonisamide - has not been established yet compared to the proven long-term practice of carbamazepine.

Absence is preferred for ethosuximide. With atypical absences or absences, combined with other types of crises, valproate and clonazepam are effective, although tolerance often develops to the latter. In refractory cases, acetazolamide is administered.

Infantile spasms, atonic and myoclonic cases of the disease are difficult to treat. Preference is given to valproate, as well as clonazepam. Sometimes ethosuximide and acetazolidamide are effective (in dosages used to treat absences). Lamotrigine is used both in monotherapy and in combination with other medicines. The effectiveness of phenytoin is limited. With infantile spasms, a good effect results in an 8-10-week course of treatment with glucocorticoids. There is no consensus on the optimal mode of glucocorticoid therapy, ACTH can be administered 20-60 units intramuscularly once a day. An auxiliary effect is provided by the ketogenic diet, however it is difficult to comply with. Carbamazepine can worsen the condition of patients with primary generalized epilepsy and with a combination of several types.

With juvenile myoclonic epilepsy, one drug (eg, valproate) is usually effective, and others (such as carbamazepine) aggravate the course of the disease; lifelong treatment.

With febrile convulsions, anti-narcotic drugs are not prescribed unless the cases of the disease are repeated after normalization of body temperature. Previously adhered to another tactic, believing that early treatment would prevent possible non-febrile seizures in the future, but the predominance of the negative effects of phenobarbital over the prophylactic result was the reason for abandoning it.

Side effects of drugs

All anticonvulsants can cause an allergic scarlet fever or a koreal-like rash, and none of them is completely safe during pregnancy.

The first year of treatment with carbamazepine is under constant control of the blood formula; with a significant decrease in the number of leukocytes the drug is canceled. With the development of dose-dependent neutropenia (the number of neutrophils is less than 1000 / μL) and the impossibility of replacing with another drug, dozarbamazepine is reduced. Treatment with valproic acid is carried out under regular control of liver function (every 3 months in the first year), and if the activity of transaminases or ammonia is increased more than two times as compared with the norm, the drug is canceled. It is permissible to increase the level of ammonia up to 1.5 times in comparison with the norm.

Admission of anticonvulsants during pregnancy is complicated in 4% by the development of fetal anticonvulsive syndrome in the fetus (the most frequent defects are harelip, wolf mouth, heart pathology, microcephaly, growth retardation, development delay, finger hypoplasia). Among the most widely used anticonvulsant drugs, carbamazepine has the least teratogenicity, and valproate is the largest. However, due to the fact that cases of the disease that continue during pregnancy often lead to the birth of birth defects themselves, treatment with anticonvulsant drugs should not be canceled. It is always useful to weigh potential benefits and risks: ethyl alcohol is much more toxic to the developing fetus than any anticonvulsant. You can prescribe folic acid, which significantly reduces the risk of neural tube defects in the fetus.

Surgical treatment of epilepsy

Approximately in 10-20% of patients, medication is ineffective. If the seizures are associated with the presence of a pathological focus, its surgical removal in most cases leads to a significant improvement or complete recovery. Since surgical treatment requires serious examination, intensive observation and high qualification of personnel, it is preferable to take place in specialized centers.

Stimulation of the vagus nerve

Periodic electrostimulation of the afferent fibers of the left vagus nerve by means of implanted pacemaker-like devices reduces the number of partial seizures by 1/3. The pacemaker is programmed, the patient independently activates it with a magnet, sensing the approach of the attack. Stimulation of the vagus nerve is used as an adjunct to anticonvulsant therapy. Side effects include impaired voice during stimulation, coughing and dysphonia. Complications are minimal. The duration of the stimulant has not yet been established.

Legal Aspects

Although epilepsy itself is not considered a mental disorder, the foregoing clearly shows that it can be closely related to a mental disorder. Mental disorder, for its part, is the basis of any protection or search for mitigants, as well as all treatment recommendations under the Mental Health Act.

At the same time, the courts in the past insisted that a severe violation of consciousness in connection with epilepsy be considered as a mental illness. This is reflected in the Sullivan case. Sullivan committed a serious act of violence and did this in a state of confusion after a case of pathology. The defense issued a statement on the automatism, not connected with insanity. However, a decision was adopted (supported by the Court of Appeal and later by the House of Lords) that it was an automatism connected with insanity, which resulted in the verdict "not guilty" because of insanity. At that time, the court had no other way out, how to apply for the isolation of Sullivan art. 37/41 of the 1983 Mental Health Act, as if he was insane in accordance with the Criminal Justice (Insanity) Act, 1964. This was one of those cases, as a result of which there are changes in the legislation. Thus, the current Criminal Procedure Act (Madness and Inability to Participate in the Trial) of 1991 grants the judge the freedom to refer the offender to the institution after establishing insanity.

One of the results of a change in the law may be a smaller meaning of the division of automatism, not associated with insanity, and the automatism associated with insanity, since at the present time the courts have other possibilities in making sentences. Consequently, under the Criminal Procedure Act (Madness and Inability to Participate in the Trial) of 1991, you can calmly claim the automatism associated with insanity and expect the referral to be treated in an appropriate institution under supervision.