EPILEPSY AND ITS SPECIAL FORMS/EPILEPSY SYNDROMES: WHAT MAY INFANTILE SPASMS OCCUR?
Infantile spasms may occur in the young child who has developmental problems of the brain or with brain damage caused by birth injury, meningitis, or head trauma. Abnormalities of metabolism such as low blood sugar or amino acid problems may also be responsible. All of these are designated as “symptomatic” infantile spasms, since they are caused by the underlying process. A second and smaller group of infantile spasms are called “cryptogenic” since their cause is unknown. Children with cryptogenic infantile spasms appear perfectly normal in development before the seizures begin.Infantile spasms are virtually always accompanied by an abnormality of the EEG known as “hypsarrhythmia,” a wildly chaotic pattern with multiple spikes and slow waves. While it may or may not be true, it is useful to think of this EEG pattern as imposing severe “static” on the brain waves so that the brain functions poorly and the child’s functioning deteriorates.A physician evaluating a child with infantile spasms should search for treatable metabolic causes of the spasms and infectious processes. An EEG and a CT or MRI scan should also be requested. Unless a specific treatable condition is found, and one rarely is, treatment for the spasms should begin promptly.We initially treat all such children with ACTH (adrenocorticotropic hormone), a form of steroid given twice a day by intramuscular injection. However, some physicians use oral steroids, benzodiazepines such as diazepam (Valium) or clonazepam (Klonopin), or valproic acid (Depakote, Depakene). The relative effectiveness of these various forms of treatment has not been tested, nor has the duration of treatment been established. Each treatment has substantial risks or side effects, but, as indicated above, there are also substantial risks in not treating this form of epilepsy. Clearly we do not understand the reason for the related retardation or its optimal treatment. Further research is needed.*94\208\8*
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