Infantile spasms is a refractory seizure disorder for which a number of different treatment regimens are available. No information is available on which treatment regimens are most widely used and which would be of practical importance in designing clinical trials to determine efficacy of new treatments. We proceeded to gather data on the most commonly employed methods of treating infantile spasms. A survey was mailed in 1991 to all junior, active, and emeritus members of the Child Neurology Society asking details about the treatment of infantile spasms. Telephone follow-up on a random sample of nonresponders was made. The total response rate was 58.3%. Most respondents who treat infantile spasms use corticotropin (ACTH) as their drug of first choice (88%). The most frequently used dosage was 40 IU per day, and the most frequent duration of treatment was 1 to 2 months. The most frequently reported side effects of ACTH or oral corticosteroid treatment were cushingism, behavior changes or irritability, hypertension, topical infections, and systemic infections. Of those not using ACTH, valproic acid was the next most commonly employed agent, followed by oral corticosteroids. The results were similar for all respondents regardless of age, sex, type of practice, number of cases of infantile spasms seen, location (United States or abroad) or whether the survey was completed by mail or telephone. These data suggest that there is a relative uniformity among child neurologists in the management of infantile spasms despite the publication of many alternative treatment strategies.

No abstract

A 3-year-old boy presented with uniocular proptosis and ophthalmoplegia. Investigation revealed a mass involving the right orbit with extension into the left orbit and paranasal sinuses, and intracranial extension involving both frontal lobes. Biopsy of an enlarged cervical node and the intranasal mass revealed esthesioneuroblastoma. This tumor has been reported rarely in a child this age, and only 12 case reports document patients under 10 years of age. The presentation as an orbital mass is previously unreported and must now be considered in the differential diagnosis of proptosis in childhood. The usual clinical, radiological, and pathological features of olfactory esthesioneuroblastoma are reviewed.

We present the findings from magnetic resonance imaging, computed tomographic scan, and single photon emission computed tomography of the brain in a 2-year-old girl with Alexander's disease. Computed tomographic scans showed prominent low-density white matter throughout the cerebral hemispheres. Magnetic resonance imaging showed increased T2 signal from the cerebral white matter but not the cerebellum or brain stem. Single photon emission computed tomography revealed diminished cerebral metabolism, particularly in the frontal regions, as compared with the cerebellum.

Infantile spasms are a seizure disorder in young infants with diverse etiologies, suggesting that they arise from any disturbance of central nervous system function during susceptible periods of development. The prognosis for normal intellectual and neurologic development parallels that of the underlying etiology. Early and appropriate treatment with ACTH may lead to seizure control in a majority of patients. The treating physician must anticipate the side effects of this modality.