Epilepsy in children and adolescents
EPILEPSY is a chronic disease of the brain, manifested by repeated convulsive or convulsive attacks (paroxysms) and specific changes in personality.
The prevalence of epilepsy in childhood and adolescence is 5-8 cases per 1000 children, which significantly exceeds the prevalence of epilepsy in adults. These figures include only patients with repeated seizures requiring medical supervision and taking anticonvulsant medications. The number of children who have had at least a single epileptic seizure is much higher and accounts for 5-7% of the child population. Epilepsy is characterized by the onset of seizures in childhood and adolescence (more than 75% of cases of epilepsy manifest in the first two decades of life).
There are three peaks of epilepsy manifestations: at the first year of life, at the age of 7-8 years and at the age of 15-16 years. The highest frequency of manifestations in the first year of life reflects the importance of exogenous damaging factors.
The causes of epilepsy are ambiguous. The main role in the origin of epilepsy belongs to hereditary predisposition. The significance of genetic factors is confirmed by a significantly higher frequency of epileptic seizures and specific changes in EEG in relatives of epilepsy patients, as well as by twin studies. The possibility of purely hereditary forms of epilepsy is now recognized, although the nature of inheritance remains unclear to date.
Organic brain damage (diffuse, local or small-focal) also plays an important role in the etiology of epilepsy. Organic brain damage and in the absence of genetic predisposition can lead to epilepsy. The delay in the occurrence of epileptic seizures after traumatic brain lesions is due to the time necessary for the formation of an epileptic focus and overcoming the mechanisms of suppression of epileptic activity of the brain. For children, the most significant brain damage during pregnancy, childbirth and in the first months of a child’s life. Of particular importance in the occurrence of epilepsy are intrauterine hypoxia and asphyxia in childbirth, often causing significant morphophysiological changes in the Central nervous system. In childhood, more often than in adults, epilepsy develops after neuroinfections. In the majority of cases of childhood epilepsy, organic damage to the Central nervous system, apparently, plays a provocative role, leading to the manifestation of genetic predisposition.
From a neurophysiological point of view, epileptic seizure is a hypersynchronous discharge of a large population of neurons, which, spreading, involves more or less extensive areas of the Central nervous system.
In the modern classification of mental diseases epilepsy is divided into idiopathic (primary) and symptomatic (secondary). In idiopathic epilepsy, the main cause of the disease is genetic predisposition. In the occurrence of symptomatic epilepsy, the main role belongs to the exogenous lesion of the Central nervous system, and genetic factors are absent or are in the background. Idiopathic epilepsy is characterized by the onset of the disease in certain age periods, the presence of a fairly well-defined specific pattern of electroencephalogram (EEG), increased epileptic phenomena of EEG during sleep and provoking effects on epileptic manifestations in EEG and intermittent photostimulation and hyperventilation (forced breathing). Idiopathic epilepsy in comparison with symptomatic differ more favorable prognosis.
At the heart of the clinical picture of epilepsy are EPILEPTIC SEIZURES (seizures, paroxysms) – suddenly occurring, short (mainly lasting from a few seconds to several minutes), clearly delineated in time pathological conditions, manifested a variety of motor, sensory, autonomic and mental symptoms. The occurrence of epileptic seizures is caused by excessive hypersynchronous discharges of significant populations of neurons in the brain. Paroxysms that determine the clinical picture of epilepsy in childhood and adolescence, are extremely diverse – from the classic short-term convulsive seizures with complete loss of consciousness or “big” tonic-clonic convulsive seizures to sufficiently long States with scene-like, colorful, painted in bright colors hallucinations and different duration of twilight States (States of darkened and narrowed consciousness) with full or partial disorientation. We will focus only on the most common epilepsy in childhood and adolescence States.
Turning off consciousness during an epileptic attack, as a rule, is preceded by an epileptic aura (a breath of wind, buckwheat.). The most common disorders during the aura are dizziness, unpleasant sensations under the spoon and in the throat, a variety of fleeting States of derealization, sensations of flashes of light, loud sounds, unusual smells or nausea, sweating, redness or, on the contrary, pale face, etc.the duration of the aura is usually from one to several seconds, sometimes even a fraction of a second. Despite this short duration epileptic aura is well remembered because of the brightness and unusual sensations. The child can not always clearly describe the state prior to the loss of consciousness, although it indicates the existence of some unusual sensations.
Large tonic-clonic seizures (grand mal, “Grand Mal”, Franz.) is the most common form of epileptic seizure. Tonic-clonic seizures can occur both during sleep and in a state of wakefulness. If the child is in a state of wakefulness after the aura, a large convulsive seizure unfolds: the patient suddenly loses consciousness and inevitably falls sharply. Often at the beginning of the attack there is a loud, piercing or somewhat compressed, like a groan cry — “epileptic cry” associated with intense tension of the muscles of the chest and abdomen and spasm of the voice slit. After several massive shudders unfolds tonic phase of the attack. Eyes open, eyeballs wound up and a few to the side. The muscles of the patient “reduces“, pulls (tonic phase lasting 30-40 seconds.). Pallor, which appeared at the beginning of the attack, is replaced by redness of the skin, quickly turning into cyanosis. In the corners of the mouth there is foam associated with excessive saliva, pronounced sweating. At the end of the tonic phase in the muscles of the limbs appear light bilateral rhythmic tremors. Then comes the next phase (clonic – duration 30-60 sec.) with sweeping convulsions in the body and limbs. During this period, there is involuntary urination, defecation, bite of the tongue, abundant foam, often pink because of the bite of the tongue and damage to the oral mucosa. Breathing in the clonic phase of an epileptic attack is intermittent, uneven, noisy. Convulsions are uniformly capture both halves of the body. At the end of the attack, the patient is in a coma for several minutes (with a complete deep loss of consciousness). Often there is involuntary urination and defecation. After a few minutes, the patient is already possible to Wake up, the dilation of the pupils disappears, superficial reflexes are restored. Clarity of consciousness returns gradually, but within 10-15 minutes the child is disoriented in the environment, speaks very slowly, does not immediately answer questions. Many children complain of headache, muscle pain, lethargy, nausea, often vomiting. After the end of the attack, patients often fall asleep; often the post-comatose state directly passes into sleep. In children in the postictal (post-criminal) period characterized by complaints of nausea and vomiting, headache, dizziness. Memories of what happened during the seizure are not preserved.
In addition to the classic large convulsive seizures in childhood and adolescence, there are often atypical rudimentary convulsive paroxysms, limited only to clonic or only tonic seizures. In some cases, there is a predominance of seizures in the right or left half of the body.
Another kind of epileptic seizures is acute, sudden, fleeting shutdown of consciousness (small seizures – petit mal, “Petit Mal”, Franz.). Most often in childhood petit mal are manifested in the form of absences (from absence – absence, Franz.), in which the main and often the only symptom of an attack is turning off consciousness against the background of normal health. Disturbance of consciousness when the absence seizure is not deep, during the attack the child is not falling, he suddenly interrupts the previous attack thing, freezes in place, not moving, stares into space, gaze empty. The child does not react to the surrounding, sometimes there is a slight swaying. Eyeballs often slightly opened up, there are sometimes light podragivanija century. The duration of the attack is usually from 1 to 20 seconds. With such short bouts, accompanied by pomeshivanii, is frequently taken for tics. The seizure stops as suddenly and rapidly as it begins. After the attack, the child immediately returns to the interrupted activity, continues the interrupted speech, without noting any changes in his health. Patients often remember the attack as a period during which they can hear or see, but can not move, respond adequately. Almost none of the children remember the beginning of the attack. Absences can be rare, but usually repeated with a large frequency, almost daily, an average of 10-15 attacks per day, in some cases up to 100-200 per day. If any motor or vegetative disorders are added to the manifestations of simple absense, then the absense is called complex (or complex). Often small seizures in children look very similar to fainting.
Propulsive seizures are characterized by forward movements of the head, torso or entire body (occasionally with a fall). A variety of propulsive seizures are nods and pecks, usually occurring in series.
With a retropulsive seizure, there is a rolling of the eyes, a deviation of the head back, throwing back the hands up and back, small clonic twitching of the eyes, head, hands, sweating and salivation, but the attack is not accompanied by the fall of the patient. Rudimentary retropulsive seizures are characterized by a slight protrusion of the eyes, small twitches of the eyeballs and eyelids.
Impulsive (myoclonic) seizures are manifested in sudden shudders and jerky movements in some muscle group, most often there is a rapid dilution or reduction of hands, which is why the child drops objects. An impulsive seizure can occur without disturbance of consciousness or with a short-term shutdown of consciousness. Usually impulsive seizures occur in the form of series (“volleys”).
Focal seizures are divided into adaptive and partial. Adversely the seizures begin with a rotation of the eyeballs, head, and further the trunk around the longitudinal axis, the patient falls, followed may experience clonic convulsions, similar to those in clonic phase of tonic-clonic seizure.
Partial (Jackson) seizures are characterized by tonic or tonic seizures in a certain group of muscles (face, upper and lower limbs), and during the attack, the seizures gradually capture the limb from its lower part to the upper (for example, from hand to shoulder). Jacksonian seizure in the possible generalization of seizures – their spreading in the whole body, and in this case, the attack is not distinguishable from a great convulsive.
In addition to large and small seizures, in the clinic of epilepsy in children and adolescents are often found uncontrolled paroxysms with confusion or without a disorder of consciousness. A special difficulty for the diagnosis (differentiation with neurotic and neurosis-like disorders) are nocturnal partial paroxysms in the form of shudders in any muscle group when falling asleep, falling out of bed in sleep, sleepwalking, collusion, including “sleepwalking”, episodes of acute paroxysmal night fear, sometimes with hallucinatory experiences, which patients then do not remember or retain extremely fragmentary memories.
Twilight state (dizziness) characterized by severe disorientation, vivid affective disorders (fear, anger, sadness) sometimes experience hallucinations when outwardly orderly behavior or frantic excitement, which may be accompanied by aggressive and antisocial actions. Twilight state suddenly begin and just as suddenly end. In this case, the patient, as a rule, completely lost memories of the period of twilight of consciousness – not only about the real events in this period, but also about their own painful experiences. But they may persist fragmentary memories of painful experiences of the period of twilight state. Short-term paroxysmal twilight States belong to the category of outpatient automatism. They are manifested in the form of automated actions (continuation of the initiated action – writing, chewing, walking with complete detachment from the surrounding, attacks of swallowing, chewing, smacking, licking lips, shaking, twisting around its axis, etc.). Often the attacks are quite complicated when, for example, the patient undresses, consistently removing their clothes and at the same time smacking his lips. Ambulatory automatism are fugues, when during the attack the patient with darkened consciousness somewhere to run, while it has not returned to consciousness, and trances in which there is sufficiently long migration, in apparently orderly behavior (for example, the child may leave or go to the other end of the city and even in another city; when he awoke, does not understand where and how we got here).
In almost all types of epileptic seizures, a so-called epileptic status can occur, which is an excessively long attack or often repeated, continuously following each other with small light intervals epileptic seizures. It is considered that attacks lasting more than 30-60 minutes are considered as epileptic status. During the epileptic status of large seizures, respiratory, cardiovascular and metabolic disorders increase, which can lead to the development of a comatose state and even death of the patient.
Along with large and small, convulsive and convulsive seizures, 30% of children with idiopathic and 60% of children with symptomatic epilepsy have a variety of mental disorders: intellectual disorders, signs of psychoorganic syndrome, asthenic symptoms, personal and neurotic reactions, psychoses.
Affective disorders in patients with epilepsy are mainly manifested in the form of paroxysmal dysphoric States, the duration of which varies from several hours to several days. Dysphoria is expressed in a vicious, sullen, unhappy mood with suspicion, unfriendliness, hostility to others and can be accompanied by aggressive behavior. Often dysphoric mood background appears shortly before the first epileptic attack and is one of the symptoms of precursors of epilepsy. In addition distonicescie painted passion you may experience of depression in which dysphoria acts as a combination of anxious, sad or fearful mood. Manic States and alternation of depressive and manic phases are much less common.
Epileptic psychoses, as a rule, arise in direct connection with attacks – in the structure of the status of convulsive attacks or in the structure of post-criminal States. The most common are episodes of twilight confusion that occurs after the seizure. At the end of twilight state can persist residual delusions, the content of which is based on the fragmentary memories of a distorted perception in the period of stupefaction. Epileptic foci in the temporal region can cause delineated States with impaired consciousness and an influx of bright visual hallucinations. During this period, the patient is completely detached from the surrounding. After leaving this state, patients retain memories of vivid hallucinatory experiences. Psychoses are most often observed in temporal epilepsy and secondary (symptomatic) generalized epilepsy. Hallucinatory paranoid (schizophrenic) symptoms observed in the localization of epileptic focus in the temporal lobe of the left (dominant) hemisphere of the brain. In General, it should be said that epilepsy is possible absolutely any productive psychopathological symptoms – from acute oneiroid psychoses to short-term, fleeting States of depersonalization-derealization, including the States of “already seen”, “already experienced”, etc., and it is, as a rule, bright, sensual and emotionally saturated character.
Characteristic changes in personality are, along with paroxysmal States, an important diagnostic criterion for epilepsy. The range of personality changes in epilepsy is extremely large, from relatively mild characterological peculiarities to disorders, evidence specific for this disease dementia. The main features of the psyche of a patient with epilepsy are stiffness, slowness of all mental processes, a tendency to get stuck on the details, thoroughness, the inability to distinguish the main from the secondary, the difficulty of switching. In the course of the disease, the slowness becomes pronounced in the mental and motor spheres. With the long course of the disease, the gestures of patients become quite stingy, facial expressions are inexpressive, devoid of liveliness. All this greatly reduces the accumulation of new experiences, especially in the child, and ultimately makes it difficult to adapt to the surrounding reality. However, changes in personality are not observed in all children with epilepsy.
A special place in the picture of epileptic changes of personality is the stuck, the viscosity of the affect, combined with explosiveness, affective explosive. These features are expressed in such characteristic features typical for epilepsy patients as vindictiveness, vindictiveness, egocentrism, malice, brutality (unstoppability of affective discharges with destructive tendencies of behavior). In addition, in children and adolescents with epilepsy, there is an increase in libido, which in combination with intense affect and explosiveness can lead to aggressive manifestations, hypersexuality, antisocial forms of behavior. Although aggressiveness and behavioral disorders occur in adolescents with epilepsy, somewhat more often than in the population, such patients are an absolute minority. For most adolescents and young men with epilepsy, characterized by hypersocial orientation, excessively strict adherence to the norms of behavior and the requirements of social responsibilities.
Patients with epilepsy tend to caricature pedantry, accuracy, thoroughness, special treatment to their family members, of religion, sometimes verging on fanaticism. Often found among the personality changes exaggerated bigotry, the sweetness, the tenderness, the combination of sensitivity, vulnerability with brutality, malice and malevolence.
Along with the described changes in personality in patients with epilepsy, asthenic and hysterical disorders are possible. Hysterical disorders are manifested both in the character of hysteria, and in individual hysterical (conversion) seizures, often existing along with seizures epileptic. Asthenic disorders are manifested in the form of rapid exhaustion, fatigue, hyperesthesia, increased excitability, sleep disorders, headaches. Epileptic dementia is based on extreme slowness and viscosity of mental processes, stiffness of thinking. Along with this, patients with epilepsy with visco-apathetic dementia have lethargy, passivity, indifference to others, spontaneity, blunt reconciliation with the disease. Reduced memory, poor vocabulary.
In patients with symptomatic epilepsy, ie, in the presence of CNS lesions, mental disorders may develop by type of psychoorganic syndrome. A slight decrease in intelligence is detected in 20-25% of patients, severe disorders-in 9-10%, gross intellectual impairment-about 1%. The development of intellectual impairment was associated with early onset epilepsy (under the age of 5 years); frequent and prolonged generalized seizures and some types of small seizures, for example, the so-called “Salamova attacks” in the form of nods with synchronous breeding in arms; the course of the disease with epileptic statuses; organic CNS lesion preceding the onset of epilepsy; inadequate antiepileptic therapy. Significant intellectual disorders are mainly found in severe forms of epilepsy in early childhood. The development and prognosis of mental disorders in epilepsy in children and adolescents depend on the severity of the disease, the form of seizures and the thoroughness and adequacy of treatment. In General, it can be argued that in most cases, epileptic seizures that began in childhood, after adolescence often stop. Most patients are characterized by a defined age period of manifestation of paroxysms; so, the attacks that occurred in infancy, most often stop after 1-2 years (especially this applies to the so-called “febrile” attacks that occur in children at an early age against the background of high temperature in viral infection); the same can be said about the seizures of the adolescent period. However, this does not exclude the need for treatment, because we can never accurately predict the favorable course of paroxysms.
With the appearance of the first seizure the child becomes irritable, Moody. Nothing satisfies him, he sleeps restlessly, loses interest in classes. At school, such a child becomes aggressive, inattentive, reacts sharply to comments and punishment, if others do not understand it. Very often in children and adolescents with epilepsy, neurotic reactions are detected, which is associated with a chronic psychotraumatic situation caused by the disease. The source of mental trauma is the experience of one’s own inferiority. Many children and especially adolescents have a painful fear of repeating the attack, especially in a public place or in front of their peers. Teenagers, patients with epilepsy, sometimes live in an atmosphere of anxious expectations of attacks and in terms of restrictions, separating it from the environment and peers do not give it to achieve the required level of socialization. Mental care is the responsibility of those around him: parents, educators, teachers, doctors, comrades. The process of school adaptation is a kind of test of adaptation to the new environment created by the disease for a child and a teenager with epilepsy.
Clinical manifestations of childhood and adolescence epilepsy have a number of features associated with the influence of age factor. For epilepsy, which began in childhood, is characterized by a predominance of generalized seizures. The most common type of seizures at school age are generalized tonic-clonic seizures, which occur in 75% of epilepsy patients as a single type of seizures or in combination with other types of seizures. Another characteristic feature is a significant number of idiopathic hereditary forms of epilepsy. A significant proportion of idiopathic forms determines a slightly better prognosis of childhood epilepsy; at the same time in early childhood manifest and the most severe cases of epilepsy, accompanied by severe mental disorders up to severe dementia. The degree of maturity of the CNS largely determines the type of attacks and other clinical manifestations of the disease. For each age period, there are the most common types of attacks, and with age, there is a modification of attacks and their transformation into attacks of another type.
To establish the fact of epileptic seizures, it is necessary to determine the exact clinical picture of the attack, the events preceding the attack, the situation in which the attacks occur, and the symptoms noted at the end of the attack. Differential diagnosis should be carried out with paroxysmal conditions of a different nature – psychogenic paroxysms, vegetative crises, fainting States of non-epileptic nature, hypoglycemic attacks, affect-respiratory attacks (in younger children) and various paroxysmal sleep disorders. Often, clinical manifestations of non-epileptic seizures may be extremely similar to epileptic seizures. In these cases, the differential diagnosis requires an assessment of the entire clinical picture in dynamics. It is necessary to accurately determine the type of attack and the form of epilepsy (since it affects the adequacy of therapy and the definition of the prognosis of the disease), the causes of attacks. A thorough examination is necessary to identify a possible organic pathology, since an epileptic attack can be a manifestation of not only epilepsy, but also of current organic diseases of the brain (including brain tumors) or a complication of many somatic diseases. You should also find out whether the child is taking any medications that can cause or provoke an attack.
The occurrence of one seizure does not yet indicate the presence of epilepsy, since it requires the elimination of organic causes and careful clarification of the conditions for the occurrence of an attack to identify possible provoking factors. Anticonvulsant therapy should be prescribed only if the doctor is fully confident in the epileptic nature of the attacks. However, it is also not necessary to delay the appointment of antiepileptic drugs, since the early appointment of therapy improves the prognosis. Constant intake of anticonvulsants (anticonvulsants) should last at least 4-5 years. Cancellation is made during the period of stable remission, after normalization of EEG. Reduction of dosages is carried out very slowly (within one to two years) under the control of EEG. It is very important to constantly convince the patient and his parents of the need for absolutely accurate implementation of doctor’s prescriptions, constant, continuous, accurate medication, perfect inadmissibility of independent change of treatment, withdrawal of drugs (sudden cancellation of anticonvulsants after their long-term use can provoke the occurrence of severe epileptic seizures), the need for regular monitoring of EEG, the desirability of compliance with an orderly regime, especially with respect to sleep.