Baby 痉挛 introduction

Introduction: Infantile spasm is also known as West syndrome, nodular epilepsy, myoclonic episodes, and large-knife-type convulsions: the syndrome has many synonymous names, such as salaam convulsion, Spasm salutation, tic salutation, eclampsia nutation, electric shock - nod - ritual convulsion (blitz-nick-salaam convulsion with two points pfe), electric shock convulsion (blitzkrieg Convulsion), systemic flexile epilepsy, flexion snoring, premature stenosis, infantile anterior flexor type, seizure convulsions, infantile myoclonic encephalopathy with abnormal rhythm. It is a serious, age-related cryptogenic or symptomatic, systemic epilepsy syndrome. It has an early onset age, a special form of convulsion, mental retardation after illness, and an electroencephalogram characterized by a peak rhythm disorder. Etiology: (1) Causes of the disease Due to the progress of the diagnosis and treatment technology, the cause of the disease is increasing. The cause of cryptogenic infantile spasm (10% to 15%) is unknown, and there are no other signs of central nervous system dysfunction. Symptomatic infantile spasm (85% to 90%) is characterized by previous signs of brain damage or a clear cause. The most common cause (78%) is cerebral ischemia and hypoxia, brain hypoplasia, intrauterine infection, and brain malformation. And innate metabolic disorders. The most common causes after birth are infection, cerebral hypoxia and head trauma. Some 71 patients with this disease were found to have abnormalities by 73%, including brain atrophy (49%), congenital abnormalities (18%), and hydrocephalus (6%). Brain malformations and calcification have also been reported. More than 20% of patients with tuberous sclerosis develop this disease. In recent years, it has been found that congenital cytomegalovirus infection, Toxoplasma infection, rubella, and herpes simplex can cause this disease. Domestic and ancestors analyzed the cause of 146 cases of this disease, 26% were primary, and the rest were symptomatic, including prenatal factors such as phenylketonuria, head deformity, cerebral palsy, Down syndrome and head deformity. Congenital brain hypoplasia; birth factors include intrauterine and intrapartum asphyxia, birth injury; postnatal factors with infectious diseases combined with encephalopathy, encephalitis, meningitis and brain trauma, vaccination. (B) pathogenesis 1. cryptogenic infantile spasm refers to no other cause of the disease, according to current knowledge and technology can not find structural or biochemical reasons. With the deepening of understanding and the advancement of diagnostic techniques, more and more cryptogenic infantile spasms will find abnormalities in brain function or anatomy to clarify the cause. 2. Symptomatic infantile spasms are caused by known brain lesions, including organic, structural lesions of the brain, or biochemical metabolic disorders. Can be divided into the following categories: (1) brain lesions: brain developmental malformations, such as neural tube development disorders, brain neuronal migration disorders, gray matter ectopic, cerebral gyrus, hydrocephalus, etc.; chromosomal diseases and congenital metabolic diseases Caused by brain development disorders; brain degenerative diseases and demyelinating diseases, such as white matter malnutrition; neurocutaneous syndrome; central nervous system infections, such as various encephalitis, meningitis, brain abscess, congenital infection, cerebral cysticercosis And other infections caused by viruses, bacteria, protozoa, parasites and fungi; cerebrovascular diseases such as intracranial hemorrhage, thrombosis, embolism, cerebral vasculitis, arteriovenous malformations, aneurysms, etc.; cerebral edema, encephalopathy, increased intracranial pressure Brain trauma, craniocerebral trauma; brain tumors, hamartomas, etc. (2) Hypoxic brain damage: diseases that cause hypoxia, such as heart and lung diseases; asphyxia; shock; convulsive brain injury. (3) metabolic disorders: congenital metabolic abnormalities, such as brain lipid deposition, abnormal glucose metabolism, abnormal amino acid metabolism, etc.; water and electrolyte disorders, such as hyponatremia, hypernatremia, hypocalcemia, low magnesium blood Symptoms; vitamin deficiency, such as vitamin B6 dependence; liver and kidney disorders; hypertensive encephalopathy; endocrine dysfunction. (4) Poisoning: drugs, metals, other chemical substances, such as lead, sputum, isoniazid, convulsions, steroids and other poisoning; drug break syndrome, such as sudden withdrawal of anticonvulsant drugs caused by epileptic state. Symptoms: The onset is within 1 year of age and the peak is 4 to 7 months. 1. The typical episode of seizures is sputum-like or nodding. It has also been observed that the disease has multiple forms of seizures, such as buckling-overextension, which is the most common, and other flexure, hyperextension, myoclonus, Loss of tension or tonicity, one side, atypical absence of seizures, etc. The form of seizures is a series of tonic sputum, which is characterized by two arms forward, the head and trunk flexing forward, and a few cases extending to the dorsal side, repeated several times or even dozens of times, sometimes accompanied by shouting or smiling. 95% of cases have mental retardation. The EEG showed a peak rhythm disorder, which was a continuous high-wave amplitude unsynchronized, asymmetric slow wave, mixed with multi-focal sharp waves and spike waves. The EEG can have a flat fast wave for a few seconds. A small number of episodes of one-sided infantile spasm, accompanied by one-sided peak rhythm disorder of EEG, were found in children with severe hemispheric injury. The causes of infantile spasms are mostly symptomatic, and may have early developmental disorders such as metabolic diseases, brain developmental malformations, neurocutaneous syndrome (nodular sclerosis, etc.) and infections. More than 80% of imaging examinations can show abnormalities such as brain atrophy and deformity. 10% to 20% is idiopathic, no cause can be found, and the prognosis is good. The differential diagnosis is mainly distinguished from benign myoclonus epilepsy in infancy, which has a typical systemic myoclonus episode, but the intelligence is normal, the EEG has a small number of spine slow waves, and the episode is easy to control. Stop before 2 years old. The prognosis is good. 2. Intelligence changes 60% to 70% of children with infantile spasm mental retardation, can increase to 85% to 90% when 2 years old. No matter whether there is mental retardation before the illness, once the sputum occurs, mental development disorders appear one after another. Diagnosis: According to the characteristics of infantile spasm, EEG peak rhythm disorder, mental movement development disorder at the initial onset can make a diagnosis of this disease. It is generally not difficult to identify the diagnosis of this disease, mainly related to other syndromes occurring at the same age. 1. Lennox-Gastaut syndrome (LGS) The onset age is usually between 1 and 8 years old, with a peak preschool age. The main symptoms are: 1 mental decline; 2 atypical loss; 3 axial tension episodes (upward, nodding or whole body). If a baby develops within 3 months of birth and has three main characteristics of infantile spasm, it is not difficult to diagnose infantile spasms. However, if the onset is after 1 year of age, then the most likely diagnosis is Lennox-Gastaut syndrome. 2. Benign myoclonus epilepsy This disease occurs in children with normal development between 6 months and 3 years old. It is characterized by systemic myoclonic convulsions, and other aspects of the cerebral interval are normal. 3. Early myoclonic encephalopathy with early Read more...