West's syndrome

Overview also known as infantile spasms syndrome, nodded eclampsia, generalized myoclonic seizures, as a subtype of epilepsy. Pathology and pathogenesis of idiopathic who accounted for 40%, were symptomatic with perinatal asphyxia, tuberous sclerosis, neocortex of styles, in addition to the phenylketonuria, maple syrup urine disease, high histidine hyperinsulinemia, Hypoglycemia and other congenital metabolic disorders. Clinical features of the behavior of infants seizures, mental retardation stagnation, EEG rhythm disorders. All in a year after the incidence. Performance for a short ankylosing cramps, a more notable flexor appearing suddenly bent neck, waist movements change, but also involve limbs. Each about a spasm ~ 15 seconds, and often occurred several times to dozens of times, and Peter bedside after the most intensive. EEG said diffuse high potential slow irregular activities, miscellaneous spikes of a sharp wave, cramps occur when short scar potential. That this attack in 2 ~ 3 years old when disappeared, but symptomatic treatment and ineffective idiopathic were growing significantly mental retardation, More than half into atypical absence seizures, or spiritual movement GTCS seizures. Differential diagnosis (1) nodular sclerosis (tuberous sclerosis) was most often chromosome Genetic that men and women should debilitating illness, and systemic each department may suffer. Facial features of the sebaceous gland tumor, seizures and intelligent receded. Skull X-ray and visible intracranial calcification ventricle wall calcification, gas cerebral angiography see the candle wall brain degeneration, CT and MRI to see multifocal-end and low-density change or calcification can be diagnosed. (2) head malformations (microcephaly) common fetal period of harmful environmental factors. Brain and the skull and development have obstacles to complete development of brain weight of not more than 1,000 grams, Trail head is not the biggest weeks more than 47 cm, and the amount of occipital flat, narrow, slightly tapered top. and development of a complete face strong control bone formation. Thickening of the scalp, the hair thicker. Most is of short stature, intellectual development stay in the idiot stage. (3) phenylketonuria (PKU) is autosomal recessive genetic, as a disease of amino acid metabolism. Characteristics of mental retardation and epilepsy, the infant is usually normal at birth, a ~ 6 months after emerging lower IQ, body or clothing can smell the special odor, stale odor or "mouse" leaves, six months after the rapid decline in IQ, epilepsy performance for infants and babies spasm syndrome or other forms of attack. Plasma phenylalanine in the 200 mg / L and above can be confirmed.