epilepsy in children
Introduction
Introduction to Pediatric Epilepsy Pediatric epilepsy, commonly known as "Yang Erfeng", is a common chronic nervous system disease in childhood, with a prevalence of 3-6. Epilepsy is an abnormal synchronous discharge of brain cell populations caused by a variety of causes, causing sudden episodes of transient brain dysfunction. Clinical manifestations are diverse and can be consciously altered or lost, limb twitching, paresthesia, and special behavior. In the past, people's understanding and research on epilepsy was less, and many patients did not receive scientific treatment, and seizure control was not satisfactory. After the 1980s, many new drugs have been released, and the level of treatment of epilepsy has been greatly improved. Epilepsy is no longer an "incurable disease." basic knowledge The proportion of illness: the incidence rate is about 0.04% Susceptible people: children Mode of infection: non-infectious Complications: cerebral edema, head injury
Cause
Causes of childhood epilepsy
(1) Causes of the disease
Etiology classification
The etiology of epilepsy is complex and diverse. The factors that constitute seizures include genetic factors, epileptic seizure factors in the brain, and inducing factors. The etiology and age factors are more obvious. Clinically, it is usually divided into the following three categories:
(1) primary epilepsy (primary epilepsy): primary epilepsy, also known as idiopathic epilepsy, is mainly determined by genetic factors, but can not find other causes, often with age characteristics. With the continuous advancement of medical science and the advent of advanced medical instruments, the detection rate of brain lesions has been greatly improved, and the diagnostic rate of primary epilepsy has been decreasing, accounting for 40% to 50% of all epilepsy. Primary epilepsy can be characterized as systemic or partial seizures, but the hereditary factors of systemic epilepsy are higher than partial epilepsy. The EEG background wave is normal, and it is a specific site-limited or bilateral symmetric synchronous epileptic discharge. Primary epilepsy is the main target of epilepsy genetics research.
(2) secondary epilepsy (secondary epilepsy): secondary epilepsy, also known as symptomatic epilepsy, refers to finding a clear cause of epilepsy. Common causes of secondary epilepsy in children include abnormal brain development such as cerebral gyrus and gray matter ectopic; brain damage caused by various causes such as perinatal injury, central nervous system infection or sequelae, head trauma, poisoning, water and electrolyte disorders, Endocrine dysfunction, hypoglycemia, vitamin deficiency, etc.; cerebrovascular diseases such as intracranial hemorrhage, endovascular inflammation, thrombosis, infarction, vascular malformations, etc.; and other metabolic, brain degeneration and systemic diseases; other genetically related metabolism Sexual diseases and syndromes often associated with epilepsy such as neurocutaneous syndrome (common nodular sclerosis, multiple neurofibromatosis and brain trigeminal angiomatosis), Rett syndrome, Angelman syndrome, mitochondrial encephalopathy, pseudo-thyroid Seizures can occur with low glandular function. This type of epilepsy can have multiple forms of clinical seizures. In addition to limited local EEG abnormalities, EEG background waves are abnormal and there is a large amount of epileptic power generation.
(3) cryptogenic epilepsy: cryptogenic epilepsy is presumed to be symptomatic, but the cause has not been found at the current level of understanding. With the deepening of brain imaging technology, it is possible to find the cause in a more advanced way in the future, or some patients may show up as the disease progresses.
The classification of seizures is helpful for the selection of clinical antiepileptic drugs and the evaluation of the efficacy of different seizures; it is helpful to study the relationship between seizure symptomology and brain structure system. The classification of epilepsy has been numerous. The classification currently used in neurology is the seizure classification proposed by the International Anti-Epilepsy Alliance (ILAE) in 1981. It is classified according to the clinical seizure form and EEG; 1989 Epilepsy and Epilepsy Syndrome Classification, in addition to the clinical onset and EEG changes, combined with the age of onset, cause and outcome.
2. Classification of seizures in 1981
(1) Partial (localized, focal) episodes:
1 simple partial seizures: sports seizures, sensory seizures, autonomic seizures, psychotic seizures.
2 complex partial seizures.
3 partial seizures evolved into systemic seizures.
(2) systemic (general, diffuse) seizures: 1 absence seizure; 2 myoclonic seizures; 3 hernias; 4 tonic seizures; 5 tonic-clonic seizures; 6 astigmatic seizures.
(3) Other seizures with unknown classification.
3. Classification of epilepsy and epilepsy syndrome in 1989
(1) Partial (limited) epileptic seizures:
1 primary disease (idiopathic): children with benign epilepsy with central-ankle spine, pediatric epilepsy with occipital discharge.
2 secondary (symptomatic) or cryptogenic: pediatric chronic progressive partial epilepsy (Kojewnikow syndrome), frontal, temporal, occipital or occipital lobe epilepsy.
(2) epileptic seizures of systemic seizures:
1 primary (esthetic):
A. Benign familial neonatal seizures.
B. Benign neonatal seizures.
C. Benign infant myoclonic epilepsy.
D. Deafness and epilepsy in children.
E. Juvenile absence epilepsy.
F. Juvenile myoclonus epilepsy.
G. Awakening when tonic - clonic epilepsy.
2 secondary (symptomatic) or cryptogenic:
A. Small infants with epilepsy encephalopathy with outbreak suppression (Datahara syndrome).
B. Infant spasm (West syndrome).
C. Lennox-Gastaut syndrome.
D. Myoclonus erects incapable epilepsy.
(3) It is not certain that epilepsy is a partial or systemic episode:
1 severe myoclonic epilepsy in infancy.
2 occurs in epileptic seizures with persistent spine slow waves during slow wave sleep.
3 Acquired aphasia (Landu-Kleffner syndrome).
(4) Epilepsy and special syndromes caused by various predisposing factors: 1 febrile seizures; 2 radioactive epilepsy; 3 others.
4.2001 seizure classification
(1) Self-limiting seizure types:
1 comprehensive sexual assault:
A. Tonic-clonic seizures (including variability that begins in the clonic or myoclonic phase).
B. Clonic seizures: no tonic components, with tonic components.
C. Typical absence of seizures.
D. Atypical absence of seizures.
E. Myoclonic seizures.
F. Tonic seizures.
G. (refers to baby ).
H. Myoclonic seizures.
I. Eyelid myoclonus:
a. Without loss of consciousness.
b. With loss of consciousness.
J. Myoclonus is out of tension.
K. Negative myoclonus.
L. Loss of tension.
M. Reflex episodes in generalized epilepsy syndrome.
2 focal episodes:
A. Focal sensory seizures:
a. manifested as simple sensory symptoms (eg, occipital lobe and parietal lobe epilepsy).
b. manifested as complex sensory symptoms (eg, epilepsy at the occipital occipital junction).
B. Focal motor seizures:
a. manifested as a simple clonic exercise episode.
b. Symptoms of asymmetry-like tonic-like movements (eg, additional motor zone episodes).
c. Behaves as a typical (temporal lobe) autonomic disorder (eg, medial temporal lobe episode).
d. manifested as hyperactivity.
e. manifested as focal negative myoclonus.
f. manifested as an inhibitory exercise episode.
C. The sneer attack.
D. Bilateral clonic seizures.
E. Secondary to a generalized seizure.
F. Reflex episodes in focal epilepsy syndrome.
(2) Type of persistent seizure:
1 comprehensive epilepsy status:
A. Comprehensive rigidity - clonic epilepsy persistence.
B. Current status of clonic epilepsy.
C. Status of absence of epilepsy.
D. Tonic epilepsy persistence.
E. Myoclonic epilepsy persistence.
2 focal epilepticus status:
A. Kojewnikow partial persistent epilepsy.
B. Persistence aura.
C. Peripheral status epilepticus (psychomotor status epilepticus).
D. Hemiplegia with convulsive side.
With the clinical application of video EEG in recent years, the episodes can be recorded simultaneously with the EEG, so new episodes are constantly being introduced. At the 24th International Conference on Epilepsy in May 2001, the International Alliance Against Epilepsy raised recommendations for seizures and diagnosis of epilepsy.
The classification of seizures in 2001 is different from the classification of seizures in 1981. The main points include: seizures are divided into self-limiting and persistent. In the scope of these two episodes, they are divided into two categories: comprehensive and focal; In focal episodes, it is no longer divided into simple and complex; in "focal sensory seizures" and "focal motor seizures", "autonomic symptoms" are no longer recognized, and autonomic symptoms are mostly Seizures accompany the phenomenon; the type of seizures increased significantly.
(two) pathogenesis
Although there are many advances in the pathogenesis of epilepsy, none of them can explain all seizures. Most people think that different epilepsy has different pathogenesis. The highly synchronized release of neurons is a feature of seizures that produce conditions that involve a range of biochemical, immunological, and genetic changes.
1. Biochemical aspects
Excitatory amino acids that cause excitatory postsynaptic potentials such as glutamate, aspartic acid and its receptor agonist N-methylaspartate, kainic acid, and gentamic ammonia Acid, etc.) increased viability; inhibitory amino acids (T-aminobutyric acid, taurine, glycine, serotonin, norepinephrine, etc.) that cause super-neuronal inhibition of inhibitory postsynaptic potential are weakened, - The decrease of aminobutyric acid receptor can enhance the excitability of cells; the toxic effect of active free radicals (O2-, OH-, H2O2, NO, etc.) on the cells of the brain; the opening of calcium channels leads to abnormal influx of Ca2, cells The decrease of the internal Ca2 binding protein causes the intracellular Ca2 to accumulate, causing cell necrosis. The flow of Ca2 into the cell is a basic condition for seizures.
2. Immunity
Immunity abnormalities such as cellular immune function are low; lack of IgA in humoral immunity, and increased production of anti-brain antibodies are potential causes of seizures.
3. Genetics
The importance of heredity in the pathogenesis of epilepsy has been confirmed by family analysis and EEG studies. The genetics of epilepsy has been confirmed to be polymorphic. The development of molecular biology technology has caused the study of epilepsy to shift from phenotype to genotype, and the ionic abnormal transmembrane transport of neuron synchronous discharge is caused by abnormal gene expression. Primary epilepsy is now known to be associated with specific genetic defects, specific ion channels and/or neurotransmitter receptors. Primary epilepsy syndrome can be caused by mutations in different genes or different mutations of the same gene. In recent years, a number of primary epilepsy loci have been identified, such as benign familial neonatal seizures autosomal dominant inheritance (AD), with type I genes on chromosome 20q13 and type II at 8q24; children with epilepsy Belonging to complex inheritance, some may be AD with high penetrance and obvious age dependence, genetic analysis and chromosome 8q24 linkage, juvenile absence epilepsy in 8q24 and 21q22; juvenile myoclonic epilepsy is also complex inheritance, part of AD is different The penetrance rate of the gene locus is 6p; the benign epilepsy of children with central-ankle sprain is also a complex inheritance or AD with age-dependent apparent rate. Several candidate genes have been studied. The mechanism for accurately describing gene defects and causing seizures is still under investigation.
Prevention
Pediatric epilepsy prevention
Instruct patients to establish good living habits, avoid excessive fatigue, lack of sleep, encourage normal learning, and avoid bad habits such as alcohol, drugs and drugs. Clinically, it is often seen that many children with epilepsy can get good control of epilepsy during normal learning, but the holiday is caused by the destruction of life rules and excessive play, especially when watching TV for a long time, playing games, etc. Significantly increased, so should pay attention to this situation if the child has increased seizures with unknown causes.
Complication
Pediatric epilepsy complications Complications brain edema brain injury
Loss of consciousness, often caused by various traumas, and even cause accidental death, convulsions can cause brain edema, brain damage.
Symptom
Symptoms of epilepsy in children Common symptoms Recurrent sacral angulation, immunodeficiency, pale, ataxia, convulsion, calcium ion, influx, nystagmus, loss of consciousness, frequent laughter
1. Partial seizure (partial seizure) Partial seizure EEG abnormal discharge limit in a certain part of the brain or from a certain part of the brain, with no disturbance of consciousness as a simple partial seizure, accompanied by a disturbance of consciousness is a complex partial seizure Partial seizures can also be generalized to systemic seizures, and EEG evolves from partial discharge to whole-brain discharge.
(1) Simple partial seizure: The consciousness of the onset of seizures is not lost, and the initial episodes can reflect the brain regions of the origin of epilepsy.
1 motor symptoms: partial seizures in childhood, including:
A. Limited motor symptoms.
B.Jackson attacks, that is, the attack starts from the side of the mouth, which in turn affects the hands, arms, shoulders, etc.
C. Torsion (rotational) episodes.
D. Half-sided seizures.
E. Diffusion is a systemic episode.
2 sensory symptoms: including:
A. Somatic sensation (numbness, pain, etc.).
B. Special feeling abnormalities (visual, listening, smelling, smelling) and hallucinations.
C. Rotational feeling, etc.
3 autonomic symptoms: including: stomach symptoms, flushing, pale, cold sweat, palpitations, vertical muscle contraction, dilated pupils and so on.
4 psychiatric symptoms: common in complex partial seizures, including cognitive disorders, memory disorders, emotional problems (fear, anger), illusions (visual enlargement, smaller) and hallucinations.
(2) complex partial seizure (complex partial seizure): conscious disorder, paroxysmal perceptual disorder, sleepwalking state, etc., often have "automatic syndrome", is an involuntary movement under the disturbance of consciousness, complex partial seizures can be from simple partiality At the onset of the attack, there is a disturbance of consciousness. It can also be a conscious disorder from the beginning. It can be seen in the epilepsy of temporal lobe or frontal lobe. EEG has a convulsion at the onset and a focal discharge in the frontal area.
(3) Partial seizures are secondary to systemic seizures: when a small infant is in a sexual assault, it is difficult to determine the level of consciousness at the time of the infant's attack, which is often expressed as:
1 Reducing reactivity: sudden reduction or stop of movement, no movement gaze or stunned, some people call "temporal pseudo absence" or "frontal absence", but not true absence of seizures .
2 automatic symptoms: common for the simple automatic symptoms of the mouth (such as pouting, chewing, swallowing, sucking and other primitive movements); or the purposeless movement of the trunk limbs, similar to normal exercise.
3 autonomic symptoms: apnea, respiratory rhythm changes, cyanosis, pale, flushing, salivation, vomiting, infant autonomic symptoms more than older children, older children rarely with autonomic symptoms as the main content of the episode.
4 convulsive symptoms: manifested as blinking, nystagmus or twitching, twisting or posture rigidity, local limbs slight convulsions, compared with older children, the attack is lighter.
2. Systemic seizures are often consciously dysfunctional, and EEG shows bilateral cerebral hemispheres symmetrically simultaneously.
(1) tonic-clonic seizure: sudden loss of consciousness during seizures, dilated pupils, generalized muscle rigidity or clonic or tonic-clonic contraction, and strong seizure refers to continuous and intense contraction of muscle groups To be characterized, the limb trunk is fixed in a certain position for 5 to 20 s, sometimes manifested as axial rigidity, head and neck back, and the torso is extremely stretched and the horn is reversed; sometimes it is characterized as "ball-like tonic attack", bowing, bending over The upper arm is lifted and the elbow is extended for 2 to 3 seconds. When standing, the attack will fall; sometimes a slight tonic attack is manifested as eyeball upturn, blinking or nystagmus, called "tonic nystagmus", clonic seizures. It refers to the rhythmic and repetitive contraction of the limbs and trunk. The tonic-clonic seizure refers to the period of tonicity, which gradually evolves into a clonic period, and finally ends.
(2) Myoclonic seizure: manifests as a rapid and powerful contraction of a certain group or groups of muscles or muscles, no more than 0.2 s. Immediately after twitching, the limbs or trunk return to their original posture (state), and the flexor ratio Extensor muscles are more susceptible, upper limbs are obvious, and there are two characteristics of myoclonus in infancy:
1 systemic large muscle myoclonus: manifested as the trunk, neck, proximal limbs suddenly violent twitching, large range of motion, isolated or continuous, EEG performance of high amplitude multi-spindle slow wave burst, or suddenly a wide range of low voltage.
2 scattered migratory myoclonus: manifested as the distal extremity, the facial group muscle group amplitude is small twitching, multi-site migration, EEG is a persistent diffuse slow wave multifocal spike, sharp wave.
(3) Disorder of tension: manifested as a sudden decrease or loss of muscle tone, unable to maintain the original posture, resulting in a sudden fall or unstable posture, sometimes the attack time is short, and the consciousness has recovered when not falling to the ground, immediately Stand up; long-term onset of tension can last for one to several minutes, showing a soft, gaze, but no motor symptoms, EEG episodes and episodes can be expressed as a full-guided slow-wave or multi-spindle slow wave; The period can also be expressed as low amplitude or high amplitude fast activity and diffuse low voltage.
(4) Absence of seizures: see the epilepsy syndrome section below.
3. Epilepsy syndrome
(1) It is only seen in childhood, and is now introduced as follows according to the age of onset:
1 benign familial neonatal seizures: autosomal dominant inheritance, often with a family history of convulsions, gene location is mostly located in 20q13.2, a few are located on the 8q chromosome, all found in full-term children, generally good at birth, after birth Within 2 to 3 days of onset, the convulsions are mainly composed of clonic sputum, which can be expressed as a limb or facial twitch, or can be expressed as a whole body clonic; a few manifestations of extensive rigidity, sometimes manifested as apnea, frequent seizures, sustained episodes The time is short, the cause can not be found from the medical history and physical examination, no special abnormalities in the EEG, biochemical examination and neuroimaging examination are normal, 10% to 14% of children converted to other types of epilepsy.
2 benign neonatal seizures: the disease is not obvious, 90% of cases occur within 4 to 6 days after birth, which is the most common on the 5th day after birth, also known as "five winds", boys slightly more than girls, this The cause of the disease is not clear, no metabolic abnormalities, and the convulsions are often manifested as clonic seizures, sometimes accompanied by apnea, frequent seizures, and sometimes epilepticus. EEG often shows sharp waves during the interictal period. Good, convulsions stop in a few weeks, no longer relapse, and mental exercise is normal.
3 small infants with epilepsy encephalopathy with outbreak suppression: In 1974, Daejeon first reported the disease, so it is also known as Otahara syndrome. It occurs within 3 months after birth, and it usually starts within 1 month, mainly for the onset of tonic. Single or a series of episodes, sometimes visible facial muscle twitching or hemiplegia, rarely myoclonic seizures, EEG manifested as "outbreak-inhibition" characteristic changes, CT and magnetic resonance examination often found brain structural abnormalities Such as brain malformation, dysplasia, etc., the treatment of this disease is difficult, a few cases are effective with ACTH, most cases have severe mental retardation and physical developmental disorders, and even early death, the survivors often evolved into infantile babies in 3 to 6 months. Clinical and EEG characteristics.
4 early myoclonic encephalopathy: the disease may be related to genetic metabolic disorders, but no obvious neuroimaging abnormalities, within 3 months after birth, family often have similar cases, mainly characterized by frequent migratory muscles There are some cases of episodes of seizures and some seizures. EEG is also characterized as "burst-inhibition". The difference with Otawara syndrome is that the outbreak-inhibition pattern of this disease is obvious during sleep, and sometimes it is clear. By the way, the prognosis of this disease is poor, anti-epileptic drugs and ACTH effects are not obvious, most of the early deaths, rarely live to 2 years old.
5 Infant sputum: It is a common epileptic syndrome, which is more common. The US data is 1/6000 to 1/4000, and the ratio of male to female is 1:2. In 1841, West first described the attack of this disease, also known as West syndrome. The disease occurs within the age of 4 to 9 months, the cause can be divided into 80% symptomatic, 20% is cryptogenic, causing multiple causes of the disease, such as various malformations caused by brain development disorders, Intrauterine infection, perinatal brain injury, nuclear jaundice, immunodeficiency, metabolic abnormalities, postnatal infection, asphyxia, chromosomal abnormalities, etc. can cause this disease, of which 10% are nodular sclerosis, the form of this disease Special, for a series of tonic attacks, can be divided into flexion type, extension type and mixed type, flexion type is nodding, bending, elbow flexion, hip flexion and other movements, stretching type is head tilting, arms straightening , knee extension and other movements, mixed performance of part of the limbs for stretching, part of the limbs for flexion, after a convulsion interval 1 ~ 2s and the second attack, can be more than 10 times or more, can be accompanied by laughter, crying, Autonomic abnormalities, EEG manifested as "high degree of disorder", The rhythm disappears, and the leads see irregular, messy, asymmetrical, high-amplitude slow waves, spikes, sharp waves, and multiple spine slow waves. This disease often combines severe mental regression or motor development, and most of the sick children change. For other forms of seizures, Lennox-Gastaut syndrome is the most common.
6 Infant benign myoclonic epilepsy: 6 months to 2 years old, the child's nerve development is normal, the episodes are short-lived myoclonus, the upper limbs extend upwards to the outside, tics can be a single episode, can also be repeated, episodes When the eyes are turned up, the consciousness is not completely lost, and it rarely affects the lower limbs. When the lower limbs have myoclonic seizures, the children can fall, and the myoclonic episodes are accompanied by abnormal discharge of EEG, which is characterized by diffuse spine or spines. Slow wave, if there is no clinical attack during waking, EEG often has no abnormal discharge, and is prone to abnormality in drowsiness and drowsiness and early stage of sleep. Sodium valproate is easy to control seizures, and some cases later turn into body tonic-clonic attack.
7 infants with myoclonus epilepsy: Dravet first described the disease in 1978, usually the first convulsions occurred at 5 to 6 months, often accompanied by fever or a history of infection or vaccination before convulsions. The clonic or tonic-clonic, after the onset of myoclonus, in various forms, can be twitching or a limb twitching, often fall when the attack, since the start of convulsions, intellectual and language development gradually lags behind or ataxia, The first year of EEG is often normal. After the second year, diffuse spikes, slow waves or multiple spines and slow waves appear. The treatment of this disease is difficult and it is difficult to control the attack.
8 Lennox-Gastaut syndrome: This syndrome accounts for 1% to 10% of pediatric epilepsy, boys are slightly more than girls, 1 to 8 years old, 3 to 5 years old, 20% of children before 2 years old, 2/ Cases of 3 may reveal abnormalities in brain structure or manifestations of hypokinesia before convulsions. The diversity of clinical seizures is characteristic of this syndrome, such as tonic seizures, atypical absence, tonic seizures, and myoclonic seizures. The latter two are not as common as the first two. The child may have several forms of seizure at the same time, and may also change from one form to another. The EEG appears to be a slower 0.5-2.5 Hz slower in the interictal period. Waves, roughly symmetric left and right, the prognosis of this syndrome is poor, treatment is difficult, convulsions continue until puberty, and even to adulthood.
9 myoclonus - standing can not attack epilepsy: 94% of sick children within 5 years of age, the most 3-4 years old, boys are significantly more than girls, myoclonus mostly for axial attacks, nodding, bending over, Both arms are lifted, often fall, can not stand, so called myoclonus - standing can not attack, EEG can be seen in the episode or interictal period, irregular spine slow wave or spine slow wave, background wave is normal, most Case treatment is better.
10 children with benign epilepsy with central-sacral spine: a common type of epilepsy in children, accounting for 10% to 20% of children with epilepsy, mostly in the 5 to 10 years old, of which 9 to 10 years old, the disease Related to heredity, there is often a family history of epilepsy, most of which occur shortly after falling asleep or before waking, manifested as oropharyngeal paresthesia and motor seizures, followed by hemifacial muscle twitching and ipsilateral upper and lower extremity twitching, sometimes developing For systemic tics, 10% to 20% of the sick children have only one episode, and another 10% to 20% of the cases have frequent episodes. The physical examination of the disease is normal, the intelligence is normal, the neuroimaging is normal, and most of the sick children EEG The background activity is normal. Spikes or spikes appear in the central or central sac, followed by a low-wavelength slow wave, which can appear alone or in clusters. Abnormal discharge increases after falling asleep. About 30% of the sick children only fall asleep. Appearance, when the child suspected of the disease, such as cerebral EEG normal, should do a sleep EEG to confirm the diagnosis, the disease has a good prognosis, most of the puberty after the onset of seizures, good response to drugs.
11 Pediatric epilepsy with occipital discharge: the age of onset is more common in 4-8 years old, the boy is slightly more than the girl, the seizure can be awake or falling asleep, the convulsions appear as a half-sided clonic seizure or expand into a whole body tonic-clonic seizure, Some patients with convulsions have visual symptoms, such as transient vision loss, dark spots in the visual field, visual hallucinations, etc., 1/3 after the attack, headache, nausea and vomiting. EEG appears as occipital and posterior iliac crest during the interictal period. High-amplitude spikes or sharp waves on one side or both sides, this abnormal discharge disappears when blinking, repeats 1 to 20s after closing the eyes, and 1/3 to 1/2 of the sick children only change after the sleep. Therefore, some cases should be done with a sleep EEG to confirm the diagnosis.
12 Acquired aphasia: This disease is also known as Landau-Kleffner syndrome. It has the highest incidence from 4 to 7 years old. There are more boys than girls. The language function is normal before onset. The aphasia is that you can hear the sound, but you can't understand the meaning of the language. Gradually developed into a language expression disorder, about half of the patients with first symptoms are aphasia, another 1/2 patients with first symptoms of convulsions, convulsions for partial seizures or systemic seizures; 17% to 25% of children with no seizures; 2/ 3 patients have obvious behavioral abnormalities, EEG background waves are normal, paroxysmal high-spine spikes in one or both sides of the sacral region, sharp waves or spine slow waves, abnormal discharge during sleep is significantly increased, in addition to general anti-epileptic drugs, Adrenal cortical hormone therapy and language training can also be applied. The prognosis of this disease is different. Most of them can control seizures, and children with small onset age have difficulty in language recovery.
13 children with epilepsy: absence is a non-convulsive seizure, 4 to 8 years old onset, 6 to 7 years old, the most girls, more girls than boys, the absence of seizures is a sudden loss of consciousness, but not fall, two Eyes gaze forward, stop the ongoing activities, after a few seconds to 1 minute or so, consciousness resumes, continue the original activities, no sleepiness or mental paralysis after the attack, frequent episodes, several times to dozens of times a day, if the absence of seizures is not controlled or Premature withdrawal, 40% combined with generalized tonic-clonic seizures, EEG showed bilateral symmetry, diffuse high amplitude 3 times per spine slow wave, excessive ventilation can induce typical EEG and clinical seizures, partial absence cases May be associated with myoclonus, dysplasia, tonic or with autodiasis, and may have different responses to drugs and prognosis.
Examine
Examination of pediatric epilepsy
Under normal circumstances, routine laboratory tests are normal, repeated convulsions, prolonged attacks can cause hypoxemia, metabolic acidosis and so on.
Necessary laboratory tests such as blood biochemistry (blood calcium, blood sugar, electrolytes and other biochemical substances), cerebrospinal fluid examination, congenital genetic and metabolic diseases, blood and urine screening tests, neuroimmunological tests, chromosome analysis and genes Positioning examination, skin and muscle biopsy, etc.
1.EEG characteristics
(1) tonic-clonic seizures: EEG features normal or non-specific abnormalities in background activity, and abnormal waves in the interictal phase can be seen in the two hemispheres with spikes, sharp waves, spine slow waves, multiple spikes, etc.; The period begins with the distribution of 10 to 20 Hz rhythmic spikes, and the amplitude is gradually increasing and the frequency is gradually slowing down; after the end of the episode, diffuse slow wave activity can be seen, and the background activity is gradually restored.
(2) Systemic large muscle myoclonus: EEG appears as a high-wave amplitude multi-spindle slow wave burst, or suddenly a wide range of low voltage.
(3) scattered migratory myoclonus: EEG is a persistent diffuse slow wave multifocal spike and sharp wave.
(4) Out-of-tension episode: EEG interictal and episodes may be characterized by a full-guided slow-wave or multi-spindle slow-wave release; the episode may also be characterized by low amplitude or high amplitude fast activity and diffuse low voltage.
(5) Infant sputum: EEG showed high degree of disorder, normal rhythm disappeared, and each lead saw irregular, messy, asymmetrical, high-amplitude slow wave, spike wave, sharp wave, and multi-spindle slow wave.
(6) Infant benign myoclonic epilepsy: Myoclonic seizures are accompanied by abnormal discharge of EEG, which is characterized by diffuse spine slow waves or multiple spine slow waves.
(7) Pediatric benign epilepsy with central-temporal spikes: most of the sick children have normal EEG background activity, and spikes or sharp waves appear in the central or central sac, followed by a low-wavelength slow wave, which can appear alone or Clusters appear.
2. EEG
(1) Benign neonatal seizures: sharp-type theta waves are often seen during the interictal period.
(2) Epileptic encephalopathy in small infants with inhibition of outbreak: EEG showed a characteristic change of "outbreak-inhibition".
(3) Early myoclonic encephalopathy: EEG also appears as "burst-inhibition", and the difference with Otahara syndrome is that the outbreak-inhibition pattern of this disease is obvious during sleep, and sometimes it is not seen.
(4) Infant benign myoclonic epilepsy: If there is no clinical attack during waking, EEG often has no abnormal discharge, and it is prone to abnormality in drowsiness and drowsiness and early stage of sleep.
(5) Pediatric benign epilepsy with central-temporal spike: abnormal discharge increases after falling asleep, about 30% of the sick children only appear after falling asleep, when the child with suspected symptoms, such as awake, the EEG is normal, A sleep EEG should be done to confirm the diagnosis.
3. CT, MRI, MRA, DSA, CT and MRI to understand brain structural abnormalities, such as brain malformations, dysplasia, etc.; PET and SPECT to understand brain function changes and help with epilepsy localization; FMRI (functional MRI), MEG (Cereography), IAP (intra-carotid artery isobaric test) and other tests to understand the relationship between brain structure and function.
Diagnosis
Diagnosis and diagnosis of childhood epilepsy
diagnosis
Complete and comprehensive diagnosis of epilepsy includes: whether it is based on clinical and EEG characteristics to determine whether it is epilepsy and the type of seizure or type of epilepsy; according to various laboratory or neuroimaging examinations to help identify the cause of epilepsy; Mental development assessment, evaluation of nervous system function.
1. Clinical data The diagnosis of epilepsy is mainly combined with medical history, clinical manifestations of various forms of seizures, with sudden, recurrent, self-remission characteristics. The current medical history should be detailed in the characteristics of the seizure, including the pre-existing incentives, aura symptoms, the location of the seizure, the nature of the seizure, the number of episodes, the consciousness at the time of onset, the condition after the episode; and the history of previous episodes and medication history, family history And developmental mileage inquiry; physical examination includes general condition, especially the characteristics related to the cause of seizures, such as special appearance, skin pigment spots (milk coffee spots, skin loss spots, head and face hemangioma) and Abnormal signs of the nervous system.
2. EEG examination EEG examination is of great value in the diagnosis and classification of epilepsy, and various paroxysmal activities such as sharp waves, spikes, sharp waves, spine slow waves, multiple spikes, and multiple spines may occur. Wave and so on. The general positive rate of conventional EEG is close to 50%; plus hyperventilation, flash stimulation and sleep EEG induction test can increase the positive rate by 20%; some multifunctional EEG, Hoter, EEG, The screen intelligent EEG monitor observes the epileptic discharge synchronized with the clinical, and increases the positive rate to over 85%. When doing EEG, pay attention to the original anti-epileptic drugs do not need to be stopped, so as not to induce seizures; EEG negative can not completely rule out epilepsy, but only EEG epileptiform discharge without clinical seizure can not be diagnosed as epilepsy.
3. Laboratory and auxiliary examination Various laboratory tests or neuroimaging examinations help to find the cause of epilepsy and evaluate the prognosis.
4. Evaluation of nervous system function In the diagnosis of childhood epilepsy, attention should also be paid to the diagnosis of abnormalities in other aspects of the nervous system and the diagnosis of concurrent diseases in various systems.
(1) Assessment of developmental quotient and IQ: Know if there is mental retardation.
(2) Various diagnostic scales: such as social life ability, child behavior, emotional disorders, memory scales, etc., find psychological and behavioral cognitive problems.
(3) Language assessment: whether there are delays in speech, developmental speech difficulties, pronunciation or dysarthria.
(4) Visual and auditory function tests: such as visual acuity, visual field, visual evoked potential, hearing test, cochlear potential map, etc., to detect perceptual impairment. Provide indications for clinical intervention therapy.
Differential diagnosis
Epilepsy is a paroxysmal disease. A considerable number of patients can be completely normal during the attack. Other tests (including neurological examination and auxiliary examination) are not necessarily abnormal. The diagnosis depends mainly on the medical history, so it is necessary to pay attention to other episodes. Identification, especially in children. Other non-seizure diseases should be identified according to clinical and EEG examinations, such as breath holding episodes, sleep disorders, syncope, habitual genital friction, multiple tics, and psychogenic episodes.
Sleep disorder
(1) Night fear or night-terrors: Night awakening often occurs within the second hour of sleep. Patients are often shocked by nightmares. After awakening, accompanied by sweating, dilated pupils, shortness of breath, palpitations and uncoordinated movements. Dream fears are common in children aged 3 to 7 years and may occur during fever, but there are no other pathological changes. The disease occurs once a month or several times a month. Sleepwalking is also a common symptom in dream fear. Sleepwalking is a benign condition in childhood, but if it is still in adulthood, it may indicate a mental disorder. Adult dreams of fear can mean feelings such as severe pain, helplessness, despair, and horror in a dream. Nightmare is easy to mix with dreams, but the two are different. Nightmares occur in the eye-opening period, and they are generally incapable of recalling dreams, even if they are incomplete. If children often have nightmares, they may not want to sleep, but they do not have this phenomenon.
(2) Somnnambulism: Sleepwalking is similar to dreaming, which is a common phenomenon. It occurs in the non-rapid eye movement and is more common in the third quarter of the whole sleep period. It is estimated that 15% of children between the ages of 5 and 12 have this phenomenon, usually more common in 5 to 7 years old. Sleepwalking is dramatic. When sleepwalking, children often sleepy, blinking slowly, having some conscious behavior, no active communication, but can be called back to bed or go back to bed to sleep. If restricted, sleepwalkers will be reluctant, but there will be no aggressive behavior. Sleepwalkers often have a dream, and sleepwalking should be differentiated from the autonomic syndrome of a complex partial seizure at night.
(3) Narcolepsy: Adolescents 10 to 20 years old are characterized by daytime sleep, squatting, sleep paralysis, and hallucinations. The occurrence of this disease is related to heredity. Both narcolepsy and epilepsy can cause loss of consciousness and stumble, and are therefore easily misdiagnosed as epilepsy. There are several different manifestations of narcolepsy. If sleep occurs during the day and is compulsive, the patient also has a loss of paroxysmal consciousness, which is confusing with the symptoms of seizures. Micro-sleeping in an awakening state is often caused by seizures of sleep or sleep apnea, which occurs repeatedly in sleep phase I or a brief outbreak occurs in the fast eye movement phase. Frequent occurrences can lead to anesthesia, where automatic behavior is seen and the patient does not remember the situation at the time of the attack. The above clinical signs have important reference significance for whether the difference is caused by the automatic behavior caused by epilepsy.
Cataplexy seizure is the sudden or partial loss of muscle tone in a patient's sudden laugh, sudden anger, or other apparent emotional effects. Patients often complain of sudden loss of muscle tone in the neck or knee. Due to skeletal muscle paralysis, patients may have difficulty breathing, close their eyes, and have difficulty speaking. If the patient with cataplexy syndrome has sudden falls and inactivity, it is easily confused with the symptoms of epilepsy, but the cataplexy syndrome is only accompanied by sudden emotional excitement, some of which retain consciousness after tripping, and no seizures. After the confusion of consciousness. Most people with squatting have one or several symptoms, usually sleep numbness or dreamy state. Sleep numbness is a symptom that occasionally occurs in normal people. Sleep sputum occurs when waking up, often accompanied by nightmares or dreams. I really want to exercise. The dreamlike state is a vivid and dramatic move that occurs in a dream. Sometimes the author himself believes that what happened is a real fact. Although healthy people may have occasional dream-like status and sleep paralysis experience, patients with catastrophic seizures may have 2 or 3 episodes in one month.
The disease requires long-term medication and comprehensive management to control excessive sleep during the day. The main drugs are methamphetamine, D-amphetamine, methylphenidate (Ritalin) and pemoline. The American Sleep Diseases Association advocates the use of small doses, which should be gradually increased to a satisfactory dose according to the patient's condition. For example, children over 7 years old, pomerin 75-150 mg per day, and methylphenidate 30-60 mg per day can obtain satisfactory results. Prognosis of the disease: Excessive narcolepsy during the day is developed within weeks or months after onset, and remains stable afterwards, with 1/3 of cataplexy, sleep paralysis and hallucinations improving with age. However, the lack of concentration caused by this disease, decreased academic performance, emotional instability and irritability are most prominent among children and adolescents. Children can behave as depression and aggressive behavior.
(4) Nocturnal pat head: This is a rare phenomenon. Some children regularly move their bodies and heads throughout the sleep process. Generally speaking, this is not a disease, but if it is used by children When the head is hit and accompanied by damage, it should be thoroughly examined to rule out conditions such as epilepsy.
(5) molar teeth: About 15% of people have sleep grinding in the age of 3 to 17, most people's night molars are temporary and benign. However, this should be distinguished from the chewing syndrome in the automatic disease at the time of seizure.
(6) nocturnal myoclonus: nighttime myoclonus is characterized by a single twitch on one or both sides of the leg, which can last for 15 to 45 seconds, with 1 twitch every 20 seconds, especially during sleep. Phase II is more common. Ticitation generally does not cause the patient to wake up, and witnessing the patient's tics can help distinguish it from epilepsy.
2. syncope
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Schott102331QT
3.(migraine)
41/4
(1);
(2)-;
(3);
(4)
203
4.(pseudoseizure)
()1982Desai4;;;93%3%-
1983Lesser10%10%20%
5.(breath holding spell)
2025s13min45
6.(crossed rub leg seizure)
1
7.(startle disease)
