Epilepsy - Information Overview

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, for many patients, existing treatment methods do not allow adequate seizure control or cause significant side effects.

An epileptic seizure is an abnormal, uncontrolled burst of electrical activity in neurons of the gray matter of the cerebral cortex that temporarily disrupts normal brain function. It is usually accompanied by a brief episode of altered consciousness with motor, sensory, and behavioral disturbances.

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Causes epilepsies

An isolated epileptic paroxysm may be triggered in healthy individuals by reversible stressors (e.g., hypoxia, hypoglycemia; fever in children). The diagnosis is made when a patient has two or more episodes of epilepsy not associated with reversible stressors.

According to etiology, epilepsy is divided into symptomatic (with a known cause, such as a brain tumor or stroke) or idiopathic (the cause is unknown). Idiopathic epilepsy may have a genetic basis.

In generalized seizures, aberrant electrical activity diffusely involves the entire cortex of both hemispheres from the very beginning, and loss of consciousness is usually noted. Generalized crises are most often associated with metabolic disorders in the brain, including those caused by genetic factors. Generalized seizures also include neonatal seizures and absences, tonic-clonic, atonic and myoclonic paroxysms.

Partial (focal) seizures often develop as a result of focal structural disorders. Pathological neuronal activity begins in one area of the cortex. Partial crises can be simple (without impairment of consciousness) or complex (with a change in consciousness, but without its complete loss). Sometimes, with a focal lesion, the excitation emanating from it so quickly covers both hemispheres of the brain that a generalized crisis immediately occurs when focal manifestations have not yet had time to develop, or a generalized paroxysm follows a short focal one (which is called secondary generalization).

Etiological factors

State	Examples
Autoimmune diseases	Cerebral vasculitis, multiple sclerosis (rare)
Cerebral edema	Eclampsia, hypertensive encephalopathy, ventricular obstruction
Cerebral ischemia	Adams-Stokes syndrome, cerebral venous thrombosis, embolic cerebral infarctions, vasculitis
Traumatic brain injury	Birth trauma, skull fracture, penetrating trauma
CNS infections	HIV, brain abscess, 4-day malaria, meningitis, neurocysticercosis, neurosyphilis, toxoplasmosis, viral encephalitis
Congenital anomalies	Genetic disorders (e.g., fifth day seizures, lipidoses such as Tay-Sachs disease), diseases associated with impaired neuronal migration (i.e., heterotopias)
Medicines	Cause paroxysms: cocaine, other CNS stimulants, cyclosporine, tacrolimus, pentylenetetrazole, picrotoxin, strychnine Lower the threshold of epileptic activity: aminophylline, antidepressants, sedative antihistamines, antimalarial drugs, some neuroleptics (eg, clozapine), buspirone, fluoroquinolone, theophylline
Extensive brain damage	Intracranial hemorrhages, tumors
Hyperthermia	Fever, heat stroke
Metabolic disorders	Usually hypoglycemia, hyponatremia; less commonly aminoaciduria, hyperglycemia, hypomagnesemia, hypernatremia
Pressure change	Decompression sickness, hyperbaric oxygenation
Withdrawal syndromes	Alcohol, anesthetics, barbiturates, benzodiazepines

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

Idiopathic epilepsy usually begins between 2 and 14 years of age. The incidence of symptomatic seizures is highest in neonates and the elderly. In children under 2 years of age, they usually result from developmental defects, birth injuries, or metabolic disorders. A significant proportion of those occurring in adulthood are secondary and are due to brain injury, alcohol withdrawal, tumors, or cerebrovascular disease; in 50% of cases, the etiology of the crises remains unknown. Cases of epilepsy in the elderly are most often due to a brain tumor or stroke. Posttraumatic seizures after traumatic brain injury with skull fractures, intracranial hemorrhage, or focal neurological defect develop in 25-75% of cases.

Cases of simulation of epileptic paroxysms by individuals with psychiatric disorders are defined as non-epileptic or pseudo-seizures.

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Pathogenesis

An epileptic seizure occurs as a result of an imbalance between excitatory and inhibitory systems in the brain. Different types of the disease are mediated by different physiological mechanisms and are associated with damage to different areas of the brain. Some antiepileptic drugs enhance inhibitory effects in the central nervous system, facilitating GABAergic transmission, while others weaken excitatory afferentation, reducing the activity of glutamatergic systems. Some antiepileptic drugs block fast neuronal discharges by interacting with sodium channels in nerve cells. Since the appearance of phenobarbital in 1912, several dozen antiepileptic drugs have been developed. To date, there is no single drug that is more effective than others, since none of them is effective in all types of crises in all situations. In this regard, the choice of drug 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 medications, other treatments, such as neurosurgery, may be effective. The ultimate goal of any treatment for epilepsy is to eliminate cases of this pathology and improve the quality of life of patients.

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Symptoms epilepsies

An epileptic seizure may be preceded by a sensory aura or mental manifestations (e.g. the smell of rotting flesh, the phenomenon of a butterfly fluttering in the stomach). Most of them end spontaneously within 1-2 minutes. Immediately after the convulsions (usually generalized), a post-seizure state occurs, the patient falls into a deep sleep, and when he wakes up, he remembers nothing, complains of general weakness, exhaustion, headaches. Sometimes Todd's paralysis (transient paralysis of the body part involved in the seizure) develops. The post-seizure state usually lasts from several minutes to an hour.

Between seizures, sufferers of this pathology usually appear neurologically healthy, although high doses of anticonvulsants suppress psychomotor reactions. Any worsening of mental or psychiatric disorders is usually due to the underlying neurological disorder that caused the development of the disease, and not to the crises as such. In rare cases, the disease is refractory to therapy (epileptic status).

Simple partial (focal) seizures

Simple partial seizures begin with specific motor, sensory, or psychomotor focal manifestations and are not accompanied by loss of consciousness. Specific symptoms indicate the area of the brain affected. In Jacksonian seizures, focal motor manifestations begin in the hand or foot and then spread to the entire limb. Some focal crises begin in the face, then the convulsions involve the arm and sometimes the leg. Some focal motor seizures manifest with the arm raised and the head turned toward the moving arm. Sometimes they become generalized.

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Complex partial seizures

A complex partial seizure is often preceded by an aura. During an epileptic episode, the patient briefly loses contact with the environment, the eyes are wide open, looking at one point; he may make automatic, aimless movements or make inarticulate sounds. He does not understand speech addressed to him and sometimes resists attempts to help him. Epilepsy lasts 1-2 minutes, the state of confusion persists for another 1-2 minutes after the seizure, but a relative understanding of what is happening appears (they purposefully avoid painful stimuli). The patient may attack a person trying to restrain him during the paroxysm, but unprovoked aggressive behavior is uncharacteristic.

When the lesion is localized in the left temporal lobe, paroxysms can cause a violation of verbal memory, when localized in the right temporal lobe - disorders of spatial visual memory. In the interictal period, patients with the temporal form of the disease more often than in the entire population experience mental disorders: serious psychological problems are detected in 33% of patients, symptoms of schizophrenia-like or depressive psychosis - in 10%. Characteristic are changes in behavior, in particular the appearance of excessive religiosity, or pronounced dependence on other people, or a tendency to hypergraphia (a writing style characterized by excessive verbosity, pedantic persistence in mentioning many unimportant details and a tendency to obsessive insertions), or changes in sexual behavior.

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Epilepsy partialis continues

This rare form of focal motor seizures usually involves an arm or half of the face; the seizures follow one another at intervals of a few seconds or minutes, appearing in periods lasting days, weeks, and sometimes even years. Epilepsia partialis continue in adults is usually caused by a structural lesion of the brain tissue. In children, it is usually a focal inflammatory process of the cerebral cortex (e.g., Rasmussen's encephalitis) due to a chronic viral infection or an autoimmune disease.

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Generalized seizures

They occur with loss of consciousness and movement disorders from the very beginning of the attack.

Infantile spasms (Salaam spasms) are characterized by sudden flexion of the arms with forward bending of the body and extension of the legs. The attacks last only a few seconds, but can be repeated many times during the day. They occur only in the first 5 years of life, and later can be replaced by other types of crises. There are usually signs of organic brain damage.

Absences (previously petit mal) are characterized by loss of consciousness for 10-30 seconds, with loss or preservation of muscle tone. The patient does not fall, there are no convulsions, but at the same time he suddenly stops all activity and resumes it after the crisis. There is no post-ictal period as such, as well as awareness of what has happened. Absences are genetically determined and occur mainly in children. Without treatment, absences are repeated many times a day, mainly in a calm environment. Paroxysms can be provoked 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 into 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 traumatic brain injury.

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

Myoclonic epilepsy cases are short, lightning-fast convulsions of one or more limbs or the trunk. They can be repeated many times, developing into a tonic-clonic crisis. Unlike other seizures with bilateral movement disorders, consciousness is not lost unless a generalized paroxysm develops.

Juvenile myoclonic epilepsy develops in childhood or adolescence. Bilateral myoclonic crises consist of single or brief arrhythmic jerks of the arms, and sometimes the lower extremities, usually in a conscious state, which in 90% of cases develop into generalized tonic-clonic seizures. The seizures are often triggered by lack of sleep, alcohol consumption, and often occur in the morning upon awakening.

Febrile seizures occur when the body temperature rises, but there should be no signs of intracranial infection. Febrile seizures occur in approximately 4% of children aged 3 months to 5 years. Benign febrile seizures are short-term, isolated, and generalized tonic-clonic. Complicated febrile seizures are focal, last more than 15 minutes, and recur two or more times during the day. Patients with febrile seizures have an increased likelihood of developing repeated afebrile seizures in the future; 2% develop the disease. The likelihood of developing and recurring cases of the disease in the future is increased in children with complicated febrile seizures, with previous neurological pathology, with the onset of paroxysms before the age of 1 year, or the presence of epilepsy in the family history.

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Status epilepticus

In epileptic status, generalized tonic-clonic seizures (two or more crises) follow each other for 5-10 minutes, and the patient does not regain consciousness in the intervals between them. The time interval of "more than 30 minutes" previously adopted for defining this nosology has been revised in order to provide medical care as quickly as possible. In the absence of assistance, a generalized seizure lasting more than 1 hour leads to persistent brain damage and can be fatal. Among the many causes that provoke its development, the most common is the withdrawal of anticonvulsants. In complex partial crises or absences, it often manifests itself as a prolonged impairment of consciousness.

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Behavior

Epilepsy is of interest to forensic psychiatrists because of its effects on consciousness (which may be associated with the commission of a crime) and its possible etiologic link to behavioral disorder (including crime) during the period between seizures.

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Aura

It is the primary focus of the attack and occurs before loss of consciousness. The subject is aware of the presence of various experiences, which are determined by the zone of discharges in the brain, and is able to subsequently recall them. Typically, an aura is characterized by involuntary movements of the limbs, discrete sensations, emotions, various hallucinations and intrusive thoughts. The aura may or may not develop into a full paroxysm.

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Complete loss of consciousness

It may be very brief, as in petit mal, or last for a few minutes, as in grand mal. A state of stupor has also been described, which may occur in petit mal as a result of rapidly succeeding episodes of the disease.

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Epileptic automatism

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

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Fugues

This behavioral disorder resembles complex epileptic automatism, but lasts much longer (several hours or days). During this time, trips may be made, purchases may be made, etc. In any case, such behavior looks somewhat strange. The subject does not retain such an event in his memory. The problem of differentiating epileptic and psychogenic fugues, which actually coincide in many ways, can cause great difficulties. A history of seizures, abnormal EEG, and the presence of fugues in the anamnesis can help here.

Twilight states

Lishman recommends that the term be restricted to episodes of prolonged anomalous subjective experiences lasting up to several hours with impaired consciousness. This is a dreamlike, absent-minded behavior and slowness of reaction. The degree of reaction to the environment can vary greatly. The subject experiences intense feelings of panic, terror, anger, or elation; he may sit quietly during the attack, but he may also have sudden outbursts of aggressive or destructive behavior. Such subjects can be very irritable and may give outbursts of rage at any attempt to interfere. This may lead to the commission of a "crime." The experiences mentioned are accompanied by disturbances in the electrical activity of the brain, often with a focus localized in the temporal region. This condition can end in grand mal.

Postictal states

After ictus, the individual may have difficulty regaining full consciousness. The subject appears confused and awkward. He is irritable, and aggressive behavior (which may lead to crime) may occur, which is usually a reaction to unwanted interference from others. Sometimes a postictal twilight state occurs, which may last from a few hours to a few days and is characterized by lethargy, hallucinations, and affective disorder, or postictal paranoid psychosis.

Interictal behavioral disturbances

The relationship between epilepsy and disrupted behaviour between seizures is complex. It may be due to changes in the brain that caused the disease, or to changes in the brain due to the severe form or to medication; it may also be a result of the psychological impact of suffering from the pathology. Any associated mental disorders or mental illnesses are also mentioned as a possible cause of disrupted behaviour between seizures.

As a result of exposure to the above factors, the subject may experience:

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

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Changes in emotional state, behavior, or personality

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Prodrome of seizures

Some subjects (most often with the temporal form of the disease) notice changes in their emotional state several hours or days before the grand mal. Usually, this is an unpleasant state with increased irritability, tension and gloomy mood. This emotional state may be associated with difficult behavior. In such a state, it is possible to commit an attack on another person.

Behavioral disorder in children

Children with some types of epilepsy (especially temporal lobe epilepsy) have been shown to be more likely than normal to exhibit antisocial behavior. Such behavior is not directly related to the seizures and is likely to be the result of a complex interaction of many factors, including brain damage, negative family influences, the type of seizure, the child's psychological response to the illness, the effect of drug therapy, and the effect of hospitalization or placement in a specialized institution. Children with petit mal are less likely to exhibit aggression than children with grand mal.

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Personality disorders in individuals

It is now generally accepted that there is no distinct epileptic personality disorder. Behavioural features previously thought to result from epileptic personality disorder are now understood to result from a combination of brain damage, institutionalisation and the effects of older generation anticonvulsants. Personality features such as aggression are more common in the temporal lobe form of the disorder. In the small proportion of individuals who exhibit personality disorder, its etiology is likely multifactorial. These factors include psychosocial influences, influences related to brain damage, abnormal electrical activity between crises and the effect of anticonvulsants.

Mental limitations

Epilepsy is much more common among individuals with mental retardation. This is a reflection of a deep-seated disorder of the brain that may underlie both conditions. It is obvious that severe seizures may lead to brain damage, which may exacerbate the subject's already existing degree of mental limitation. Among individuals with severe mental retardation, 50% had a history of epileptic seizures. However, if brain damage is excluded, the children's intelligence is within normal limits.

Sexual dysfunction

A number of studies consistently describe decreased libido and impotence in individuals. However, if we exclude the assumption of decreased levels of male sex hormones, a direct connection between the disease and sexual dysfunction is not accepted by specialists. Hypersexuality is rarely noted. In some rare cases, a connection with temporal epilepsy, fetishism and transvestism is shown. The literature describes cases claiming that surgical removal of the lesion in the temporal region cured fetishism. However, it is unclear whether there was really a direct connection with the temporal form of the disease or whether sexual dysfunction was a consequence of distorted human relationships due to the subject.

Crimes

In the 19th century, epilepsy or a tendency to it was considered a feature of many criminals. Moreover, according to the concepts of that time, crimes committed in blind rage were also considered a manifestation of the epileptic process. Modern research refutes this point of view. Studies of patients visiting outpatient clinics did not find excessive criminality in them. At the same time, a more complete study by Gudmundsson of all Icelanders revealed a slight increase in criminality 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, no convincing evidence was obtained for the commission of a crime in a state of automatism, although ten people committed crimes immediately before the onset of a seizure or immediately after its end. In a study of 32 people in special hospitals, two may have been in a state of post-confusion at the time of committing a crime. That is, although epilepsy may indeed be a factor leading to antisocial behavior in some cases, in general this connection is not expressed among individuals, and crimes are rarely committed during a crisis.

1. The crime may occur in a disturbed state, the cause of which is the paroxysm itself. This happens rarely.
2. The crime and the attack could be a coincidence.
3. Brain damage due to epilepsy may have caused personality problems that resulted in antisocial behavior.
4. The subject may develop a strong antisocial attitude towards phenomena as a result of the difficulties he experiences in life due to illness.
5. A deprivation environment in early childhood can both give rise to an antisocial attitude towards phenomena and expose the subject to the influence of epileptogenic factors.
6. Antisocial individuals are more likely to be in dangerous situations and suffer more head injuries that can cause illness than normal.

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EEG changes and violent crime

Violence is unlikely to be a common occurrence in direct association with seizures. Typically, any seizure-related violence occurs in the post-confusion state and involves attacks on people who somehow interfere with the situation. Violence can also occur (very rarely) in epileptic automatism. It has also been described in association with amygdala discharges. Most violence by individuals occurs between seizures. Reviews of research on the increased prevalence of violence in individuals with the disease have yielded mixed results. For example, in a study of 31 subjects with temporal lobe epilepsy referred to a clinic, 14 had a history of aggression. Violence was usually mild and did not correlate with EEG or CAT scans. However, such behavior was correlated with male gender, the presence of behavioral disorders from childhood (which often led to education in special residential institutions), personality problems in adulthood, and poor intelligence. And, of course, violence can occur in cases of psychosis.

It has also been suggested that EEG changes are more common in violent offenders. This view is based on a classic study that found that EEG abnormalities were more pronounced if the murder was impulsive or unmotivated. Wiliam argued that impulsive men with violent tendencies had higher levels of temporal lobe abnormalities. However, these findings have not been confirmed by other researchers and should be treated with considerable caution. Gunn and Bonn, for example, found no association between temporal lobe epilepsy and violence. Lishman's study of individuals with head trauma confirmed that frontal lesions were most often associated with aggression. Driver et al. failed to find significant differences between the EEGs of murderers and those of individuals without violent tendencies unless the EEG examiner had prior information about the individuals.

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Evaluation of the offender

Fenwick proposed the following six criteria to help psychiatrists determine the degree of veracity of a subject's statement that his crime was committed in a state of impaired consciousness.

1. It should be known about the patient that he suffers from this pathology, that is, this should not be his first attack.
2. The action taken must be uncharacteristic of the person and inappropriate to the circumstances.
3. There must be no signs of intent or attempts to conceal the crime.
4. Witnesses to a crime must describe the state of the offender's impaired state, including a description of the subject when he suddenly becomes aware of what is happening and his confusion at the moment the automatism ceases.
5. There must be amnesia for the entire period of automatism.
6. There should be no memory impairments preceding 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 cannot prove or exclude the presence of automatism.

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Forms

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

Generalized in turn is subdivided into primary generalized with two different forms - grand mal and petit mal, and secondary generalized, which is observed when focal epilepsy captures the thalamocortical pathways, thus causing a generalized paroxysm. This can lead to grand mal with a preceding aura.

Grand mal is characterized by a tonic phase, followed by a clonic phase and a period of unconsciousness lasting several minutes. In petit mal, there are only moments of loss of consciousness, and the patient immediately resumes normal activity. Absence is manifested to an outside observer by a transient "blank" facial expression and, possibly, slight twitching of the limbs or eyelids, an akinetic seizure, expressed by a sudden fall, and a myoclonic jerk of the outstretched limb.

In focal (partial) epilepsy, such attacks begin in a part of the cerebral cortex. The symptoms depend accordingly on the area of the brain involved. If only part of the brain is involved, a conscious sensation (aura) may be present. The nature of the sensation provides a clue to determining the discharge zone. The focal form is in turn subdivided into simple partial (focal) paroxysms without affecting consciousness and complex partial (focal) attacks with complex movements and impaired consciousness (mainly occurring in the temporal region).

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Diagnostics epilepsies

First of all, it is necessary to make sure that the patient had an epileptic paroxysm, and not a fainting spell, 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 in the intensive care unit is indicated, if the diagnosis was established earlier, in an outpatient setting.

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Anamnesis

The presence of aura, classic epileptic seizures, as well as tongue biting, urinary incontinence, prolonged loss of consciousness and confusion after the crisis indicate an epileptic seizure. When collecting the anamnesis, information should be obtained on the first and subsequent crises (duration, frequency, sequence of development, the longest and shortest interval between seizures, the presence of aura and post-ictal state, provoking factors). It is necessary to identify potential causes of symptomatic epilepsy (previous traumatic brain injury or CNS infection, existing neurological disorders, drug use or withdrawal, violation of the anticonvulsant regimen, the presence of seizures or neurological disorders in the family history).

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Physical examination

Physical examination findings are almost always normal in the idiopathic form, but may be severe in the symptomatic form. Fever and neck stiffness should raise concerns about meningitis, subarachnoid hemorrhage, or encephalitis. Congestive optic discs indicate increased intracranial pressure. Focal neurologic deficits (eg, asymmetry of reflexes or muscle strength) suggest a structural lesion in the brain (eg, tumor). Skin lesions may be seen in neurocutaneous disorders (eg, axillary or café-au-lait spots in neurofibromatosis, hypopigmented skin macules or shagreen plaques in tuberous sclerosis).

Study

For patients with an established diagnosis and no abnormalities according to neurological examination data, only determination of the concentration of anticonvulsant drug in the blood is indicated, provided that no signs of traumatic brain injury or metabolic disorders have been detected.

If this is the first seizure in a patient's life or if pathology is detected in the neurological examination, an emergency CT scan of the brain is indicated to exclude focal lesions or hemorrhage. If CT does not reveal any changes, MRI is performed due to its better resolution in detecting tumors and abscesses of the brain, cerebral venous thrombosis and herpes 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 levels and liver enzymes). If meningitis or CNS infection is suspected, CT scan of the brain and lumbar puncture are performed if CT shows no abnormalities. EEG allows diagnosing epileptic status in complex partial paroxysms and absences.

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

However, the diagnosis is made on the basis of the clinical picture and cannot be excluded with a normal EEG. In rare attacks, the probability of EEG confirmation of epilepsy decreases. In patients with a confirmed diagnosis, the first EEG does not show pathological changes in 30% of cases; the second EEG, performed after sleep deprivation, reveals pathology in only 50% of cases. Some patients never have pathological changes on the EEG.

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

Treatment epilepsies

The optimal approach is to eliminate possible causes of symptomatic seizures. If no potential cause can be identified, anticonvulsants are indicated, usually after the second episode of epilepsy. The appropriateness of prescribing anticonvulsants after one (sometimes only) crisis is controversial, and the potential risks and benefits should be discussed with the patient.

During a seizure, the primary goal is to prevent injury. Loosen the neck from tight clothing and place a pillow under the head to prevent aspiration. Avoid trying to prevent tongue injury, as this can cause damage to the patient's teeth or the fingers of the person providing assistance. These measures should be communicated to family members and co-workers.

Until adequate control of the disease is achieved, certain activities in which loss of consciousness could be life-threatening (driving, swimming, climbing mountains, bathing) should be avoided. Once complete control is achieved (usually more than 6 months), such activities are allowed, provided that precautions are taken (e.g., in the presence of someone). A healthy lifestyle with moderate physical activity and participation in social activities is encouraged. Some cases are subject to notification (e.g., to transport control, in accordance with local legislation), although if there are no pathological events for 6-12 months, the patient may be allowed to drive a vehicle.

It is recommended to avoid alcohol and drugs, as cocaine, phencyclidine and amphetamines can provoke crises. It is also advisable to exclude all drugs that lower the seizure threshold (in particular, haloperidol, phenothiazine).

Family members need to develop a reasonable line of behavior towards the patient. Excessive concern, which gives rise to a feeling of inferiority, is better replaced by support and sympathy, which allows overcoming these and other psychological problems, which prevents additional disability of the patient. Inpatient psychiatric care is indicated only for serious mental disorders or frequent severe attacks that do not respond to pharmacotherapy.

First aid

Most cases of pathology resolve on their own within a few minutes and do not require emergency drug therapy.

Emergency intervention is required to stop status epilepticus and crises lasting more than 5 minutes, while monitoring respiratory parameters. If there are signs of airway obstruction, the patient is intubated and, having provided intravenous access, lorazepam is administered at a dose of 0.05-0.1 mg/kg at a rate of 2 mg/min. If necessary, the dose is increased. If the case of epilepsy cannot be stopped after the administration of 8 mg of lorazepam, fosphenytoin is additionally administered at a dose of 10-20 EF (phenytoin equivalents)/kg intravenously at a rate of 100-150 EF/min; the second-line drug is phenytoin - at 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 fosphenytoin or 5-10 mg/kg of phenytoin are additionally administered. Persistence of seizures after administration of lorazepam and phenytoin indicates refractory status epilepticus, which requires administration of third-line drugs - phenobarbital, propofol, midazolam or valproate. Phenobarbital is administered at a dose of 15-20 mg/kg intravenously at a rate of 100 mg/min (for children at 3 mg/kg/min); if cases of pathology continue, additional phenobarbital should be administered at a rate of 5-10 mg/kg or valproate at a dose of 10-15 mg/kg intravenously. If status epilepticus cannot be relieved after the above measures, the patient is intubated under general anesthesia. It is difficult to recommend an optimal anesthetic, but in most cases preference is given to propofol - 15-20 mg/kg at a rate of 100 mg/min or phenobarbital at 5-8 mg/kg (initial dose) followed by infusion of 2-4 mg/kg/h until signs of activity on the EEG are suppressed. Inhalational anesthetics are rarely used. After the epileptic status has been relieved, its cause is first identified and eliminated.

Prescription of anticonvulsants for prophylactic purposes is advisable for patients who have suffered a traumatic brain injury with skull fractures, intracranial hemorrhage or focal neurological symptoms. The use of anticonvulsant therapy reduces the risk of pathology cases during the first week after the injury, but does not prevent post-traumatic epilepsy several months or years later. If there were no attacks in the acute period of injury, anticonvulsant therapy is discontinued after 1 week.

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Long-term drug treatment

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

In 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 over 1-2 weeks to the standard therapeutic level (based on the patient's body weight), while simultaneously assessing tolerance to the drug. After approximately a week of treatment with a standard dose, the concentration of the drug in the blood is determined. If the patient continues to have seizures at a subtherapeutic level, the daily dose is gradually increased. If the patient develops symptoms of intoxication, and cases of pathology continue, the dose is reduced and a second drug is gradually added. When treating with two drugs, special care is needed, since their interaction may increase the toxic effect as a result of slowing down their metabolic degradation. Then the dose of the ineffective drug is gradually reduced until complete withdrawal. If possible, it is recommended to avoid polypharmacy and not to prescribe several anticonvulsants simultaneously due to the increased frequency of side effects and drug-drug interactions; prescribing a second drug helps approximately 10% of patients, while the frequency of side effects more than doubles. Additional drugs can increase or decrease the concentration of the main anticonvulsant, therefore, when selecting therapy, the doctor should first consider aspects of drug-drug interactions.

Once the selected drug has completely stopped the cases of the disease, it is necessary to continue taking it without interruption for 1-2 years, after which the drug can be tried to be discontinued, reducing the dose by 10% every 2 weeks. Approximately half of the patients do not experience further crises without treatment. Relapse is most likely if cases of epilepsy have been observed since childhood, if more than one drug was required to control the paroxysms, if the seizures continued against the background of anticonvulsant therapy, if the cases of the disease are partial or myoclonic, and also if the patient has encephalopathy or pathological changes in the EEG during the previous year. In these cases, relapse within the first year after discontinuation of anticonvulsant therapy occurs in 60%, and during the second year - in 80% of patients. If seizures were poorly controlled by drugs from the very beginning, recurred when trying to discontinue treatment, or are extremely undesirable for the patient due to social reasons, then treatment should be carried out indefinitely.

Once a drug reaction has been established, blood levels are much less important to the physician than the clinical course. Some patients will already exhibit toxic effects at low blood levels, while others tolerate high doses well, so monitoring concentrations serves only as an auxiliary guide for the physician. An adequate dose of any anticonvulsant is the lowest dose that completely stops seizures with minimal side effects, regardless of its blood concentration.

In generalized tonic-clonic crises, the drugs of choice are phenytoin, carbamazepine, and valproic acid (valproates). For adults, the daily dose of phenytoin is divided into several doses or the entire dose is prescribed at night. If epilepsy does not stop, the dose can be gradually increased to 600 mg/day under monitoring of the drug content in the blood. At a higher daily dose, dividing it into several doses helps to reduce the toxic effects of the drug.

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

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

Infantile spasms, atonic and myoclonic cases of the disease are difficult to treat. Preference is given to valproates, as well as clonazepam. Sometimes ethosuximide and acetazodamide are effective (in dosages used to treat absences). Lamotrigine is used both as a monotherapy and in combination with other drugs. The effectiveness of phenytoin is limited. In infantile spasms, a good effect is given by an 8-10-week course of glucocorticoid treatment. There is no consensus on the optimal glucocorticoid therapy regimen; ACTH can be administered intramuscularly at a dose of 20-60 units once a day. A ketogenic diet has an auxiliary effect, but it is difficult to adhere to. Carbamazepine can worsen the condition of patients with primary generalized epilepsy and with a combination of several types.

In juvenile myoclonic epilepsy, one drug (eg, valproate) is usually effective, while others (eg, carbamazepine) worsen the disease; treatment is lifelong.

Anticonvulsants are not prescribed for febrile seizures unless cases of the disease recur after normalization of body temperature. Previously, a different tactic was followed, believing that early treatment would prevent possible non-febrile seizures in the future, but the prevalence of negative effects of phenobarbital over the preventive result was the reason for abandoning it.

Side effects of medications

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

The first year of treatment with carbamazepine is carried out under constant monitoring of the blood count; if the number of leukocytes decreases significantly, the drug is discontinued. If dose-dependent neutropenia develops (neutrophil count less than 1000/mcl) and it is impossible to replace it with another drug, the dose of carbamazepine is reduced. Treatment with valproic acid is carried out under regular monitoring of liver function (every 3 months during the first year), and if transaminase activity or ammonia content increases more than twice as much as normal, the drug is discontinued. An increase in ammonia levels up to 1.5 times as much as normal is acceptable.

Taking anticonvulsants during pregnancy is complicated by the development of fetal anticonvulsant syndrome in the fetus in 4% of cases (the most common defects are cleft lip, cleft palate, cardiac pathology, microcephaly, growth retardation, developmental delay, and finger hypoplasia). Among the most widely used anticonvulsants, carbamazepine has the least teratogenicity, and valproates have the greatest. However, since cases of the disease that continue during pregnancy often lead to congenital defects, treatment with anticonvulsants should not be discontinued. It is always useful to weigh the potential benefits and risks: ethyl alcohol is much more toxic to the developing fetus than any anticonvulsant drug. Folic acid can be prescribed, which significantly reduces the risk of neural tube defects in the fetus.

Surgical treatment of epilepsy

In approximately 10-20% of patients, drug treatment is ineffective. If attacks are associated with the presence of a pathological focus, its surgical removal in most cases leads to significant improvement or complete recovery. Since surgical treatment requires a thorough examination, intensive monitoring and highly qualified personnel, it is preferable to undergo it in specialized centers.

Vagus nerve stimulation

Periodic electrical stimulation of the afferent fibers of the left vagus nerve using 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 an attack. Stimulation of the vagus nerve is used as an adjunct to anticonvulsant therapy. Side effects include voice disturbance during stimulation, coughing and dysphonia. Complications are minimal. The duration of the stimulator's action has not yet been established.

Legal aspects

Although epilepsy itself is not considered a mental disorder, the above clearly shows that it may be closely associated with a mental disorder. Mental disorder, for its part, is the basis of any defence or search for mitigating factors, and of all recommendations for treatment under the Mental Health Act.

However, the courts have in the past insisted that severe impairment of consciousness due to epilepsy be treated as a mental illness. This was reflected in the Sullivan case. Sullivan committed a serious act of violence and did so in a state of confusion following an episode of pathology. The defence pleaded automatism unrelated to insanity. However, it was decided (upheld by the Court of Appeal and later the House of Lords) that it was automatism related to insanity, resulting in a verdict of not guilty by reason of insanity. The court had no choice at the time but to apply s. 37/41 of the Mental Health Act 1983 to isolate Sullivan as if he were insane under the Criminal Justice (Insanity) Act 1964. This was one of those cases that results in changes in the law. Thus, the current Criminal Procedure (Insanity and Incompetence) Act 1991 gives the judge discretion to commit an offender to an appropriate institution after a finding of insanity.

One effect of the change in law may be to make the distinction between automatism unrelated to insanity and automatism related to insanity less important, as the courts now have other options when passing sentence. It is therefore safe to allege automatism related to insanity under the Criminal Procedure (Insanity and Incompetence) Act 1991 and expect to be sent to an appropriate institution for supervised treatment.