Abstract
West syndrome is including in the spectrum of encephalopathies of early beginning in lactation and childhood and is characterized by: spasm, psychomotor delay and hypsarrhythmic electroencephalogram, probably bound to factors of neurological development. Its prevalence is about 0, 15 to 0, 2/1000, in children under ten years old. When the causes are prenatal the spasm appears in the first three months of life, and those of other cause between nine months and two years of age. They are characterized by brief contractions in flexion, extension or both, generally are bilateral, symmetrical and two-phase. The duration of spasm varies from an isolated two seconds crisis, in the beginning, to one hundred spasms salve later. Epileptic seizures are the first manifestation of the syndrome, but the psychomotor delay can precede them, when initiating the spasms a development regression is evident.
There is no sufficient evidence to determine the optimal dose of ACTH. High doses of 80-120 UI are not more effective than those of 20-40 UI. Prednisolone 2 mg/k/d, and hydrocortisone 15 mg/k/d by a month have had good effects. Vigabatrin is the more used antiepileptic medication. The crises yield in the first days of treatment, whereas the hypsarrhythmic pattern can delay 2-4 weeks more. When Down’s syndrome is the cause of the West syndrome pyridoxine has given good results. The surgical extirpation of epileptogenic focus, like cortical dysplasia, tumors, etc, has demonstrated utility, but it is not easy to identify epileptogenics areas before the 18 months even with positron emission tomography (PET).
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