early infantile epileptic encephalopathy
Introduction
Introduction to early infantile epilepsy encephalopathy Early infantile epilepsy, also known as infantileepilepticencephalopathy, is a malignant epilepsy. The disease has an early onset age, EEG has an outbreak-inhibition wave pattern, convulsion is difficult to control, and the prognosis is poor, which can be transformed into infantile spasm. basic knowledge The proportion of illness: 0.002% Susceptible people: infants Mode of infection: non-infectious Complications: infantile spasms
Cause
Early infantile epilepsy encephalopathy
(1) Causes of the disease
The etiology of this disease has not been clarified, and may have a family history of epilepsy. Presumably, the cause is a family history of epilepsy, a severe pregnancy-induced pregnancy syndrome in the third trimester of pregnancy, a history of early anesthesia in the first trimester of pregnancy, and a history of postpartum asphyxia in the child.
(two) pathogenesis
At autopsy, severe neuronal necrosis is observed. The burst suppression waveform is related to this, including early myoclonic encephalopathy and Ohtahara syndrome, all of which are symptomatic epilepsy. Most of Ohtahara syndrome has severe congenital or perinatal brain damage, nerve Imaging studies often find more obvious structural abnormalities. Early myoclonic encephalopathy often has a family history of similar lesions, suggesting that it is associated with hereditary metabolic disorders.
Prevention
Early infantile epilepsy prevention
Prevention of pediatric epilepsy should start from many aspects. The prevention of symptomatic epilepsy should pay attention to the following aspects:
1. Pay attention to perinatal health care to protect the fetus and newborn from hypoxia, birth injury, infection, etc., especially to prevent neonatal asphyxia and hypoxic ischemic encephalopathy.
2. Active prevention and treatment of febrile seizures should pay enough attention to the febrile seizures in infants and young children, try to prevent seizures;
3. Actively prevent the timely treatment of various diseases of the nervous system in children and reduce the sequelae.
4. Prevention of biochemical metabolic disorders.
5. Do a good job of genetic counseling For some serious hereditary diseases that cause epilepsy, genetic counseling can be carried out, and some can be used for prenatal diagnosis or newborn screening to determine the necessity of stopping pregnancy or early treatment.
Complication
Early infantile epilepsy encephalopathy complications Complications, infantile spasms
Frequent seizures or infections, some cases can be converted to infantile spasm or further to Lennox-Gastant syndrome, survivors often have severe brain damage, mental retardation, language barriers, hemiplegia or cerebral palsy, denervated or cortical Wait.
Symptom
Symptoms of early infantile epilepsy encephalopathy Common symptoms Linguistic retardation Intelligence reduces the body's rigidity of epilepsy... The cingulate gyrus of frontal lobe epilepsy
1. Attack onset within 3 months, tonic and/or tonic-clonic seizures, can occur from 2 to 40 times per day, each episode is short, short 10s, long only 5min, can be a string of attacks, Some sick children later turned into infantile spasms. The main type of early myoclonic encephalopathy is systemic or migratory myoclonus, which generally does not translate into infantile spasms.
2. Psychic dyskinesia mental and physical development is significantly backward, severe people will never cry and gaze, the function of the girders is lost after the disease, the survivors often do not look up, language barriers, limb hemiplegia, etc., the degree and episodes of psychomotor disorders The starting age is related.
Examine
Early detection of infantile epilepsy encephalopathy
No special results, such as the cause and genetic disorders, such as non-ketotic hyperglycemia, propionation, etc., may have the corresponding laboratory test results change.
1. CT examination mostly has different degrees of cortical atrophy, some of the left frontal lobe low density shadow, the midline structure left shift, ventricular dilatation and so on.
2. EEG features a characteristic outbreak suppression EEG. According to the characteristics and evolution of EEG, this syndrome can be divided into type I and type II. Type I EEG can evolve from continuous outbreak inhibition. The rhythm is disordered and then converted into a broad slow-spinning slow wave; type II is a lesion-like spike that evolves from an outbreak suppression wave.
Diagnosis
Diagnosis and diagnosis of early infantile epilepsy encephalopathy
Diagnostic criteria have mandatory and reference conditions, which are described below.
1. Diagnostic prerequisites
(1) The age of onset is neonatal and small infants (mainly within 3 months after birth).
(2) frequent and uncontrollable tonic and/or tonic-clonic seizures.
(4) Characteristic outbreak suppression type EEG.
(4) Severe psychomotor disorders.
2. Diagnostic reference conditions
(1) Family history, multiple causes.
(2) can be converted into infantile spasms.
Daejeon syndrome and infantile sputum (West syndrome), Lennox-Gastant syndrome is an age-dependent epileptic encephalopathy.
(3) The commonalities of the three are:
1 age dependent;
2 frequent and difficult to control episodes;
3 persistently abnormal abnormal EEG;
4 a variety of causes;
5 mental damage;
6 poor prognosis, the three have their own clinical and EEG differences, and suggest age-related changes, Otahara syndrome can be converted to West syndrome at 4 to 6 months, West syndrome in It turns into Lennox-Gastant syndrome from 1 year and a half to 3 years old.
Differential diagnosis
In the differential diagnosis and differentiation of West syndrome, Lennox-Gastant syndrome, attention must be paid to their different clinical features, the strong and straight or clonic seizures of Daejeon syndrome; the short head of West syndrome, the flexion of the limb to the trunk Myoclonic seizures; triads of Lennox-Gastant syndrome (myoclonic seizures, tension or tonic-clonic seizures, atypical absences), and outbreaks of Otahara syndrome inhibit EEG; peak rhythm of West syndrome Disorder graph; diffuse slow spine slow wave waveform of Lennox-Gastant syndrome, both of which have characteristic and differential diagnostic significance.
