Objective. To compare the efficacy of corticotropin (ACTH) (150 U/m2/day) and prednisone (2 mg/kg/day), given for 2 weeks, in suppressing clinical spasms and hypsarrhythmic electroencephalogram (EEG) in infantile spasms (IS). ACTH and prednisone are standard treatments for IS. ACTH at high doses causes severe dose- and duration-dependent side effects, but may be superior to prednisone, based on retrospective or uncontrolled studies. Blinded prospective studies have shown equal efficacy of prednisone and low-dose ACTH, and low versus high-dose ACTH. Design. A prospective, randomized, single-blinded study. Subjects and Methods. Patient population consisted of consecutive infants fulfilling entry criteria, including the presence of clinical spasms, hypsarrhythmia (or variants) during a full sleep cycle video-EEG, and no prior steroid/ACTH treatment. Response required both cessation of spasms and elimination of hypsarrhythmia by the end of the 2-week treatment period, as determined by an investigator "blinded" to treatment. Treatment of responders was tapered off over 12 days; those failing one hormone were crossed-over to the other. Results. Of 34 eligible infants, 29 were enrolled. Median age of patients was 6 months. Twenty-two infants were "symptomatic" with known or suspected cause, and seven were cryptogenic (two normal). Of 15 infants randomized to ACTH, 13 responded by both EEG and clinical criteria (86.6%); seizures stopped in an additional infant, but EEG remained hypsarrhythmic (considered a failure). Four of 14 patients given prednisone responded (28.6%, with complete clinical-EEG correlation), significantly less than with ACTH, (x2 test). Conclusions. Using a prospective, randomized approach, a 2-week course of high-dose ACTH is superior to 2 weeks of prednisone for treatment of IS, as assessed by both clinical and EEG criteria.

This study investigated the potential influences of general processing capacity and sustained selective attention on the temporal processing of a group of children with specific language impairment (SLI) and a group of age-matched (CA) controls. Children completed a sustained selective auditory attention task and two speech processing tasks, the Speech Identification Task, representing a cognitively more complex task, and a standard speech discrimination task, representing a cognitively less complex task. The speech stimuli included two-formant, 40-ms transition [ba] and [da] consonantvowel (CV) syllables and the nonstop CV syllable [sa]. We hypothesized, in accordance with Tallal's temporal processing deficit view, that if SLI children have a fundamental deficit in temporal processing, they should demonstrate poor performance on both speech processing tasks relative to CA children. By contrast, if the temporal processing problem of SLI children relates to a limitation in general processing capacity, the SLI children should show better performance on the discrimination task compared to the identification task. Alternatively, if poor sustained selective attention mediates SLI children's poor temporal processing, the SLI children should show poor performance on an independent measure of auditory attention, which in turn should account for a portion of the variance in any observed group differences in temporal processing. Results showed no group difference in sustained selective auditory attention. On the identification task, group, stimulus-type, and interaction effects emerged. SLI children performed more poorly than CA children, and stop CV syllables were identified less frequently than the nonstop CV syllable. On the discrimination task, there were no main effects or interactions for accuracy (A′ score). For the reaction time analysis, only a stimulus-type effect was found, with children responding faster to the /sa/ syllable. Results were interpreted to suggest that these SLI children did not evidence a basic temporal processing deficit. Rather, the SLI children's poor identification task performance was interpreted to reflect an interaction between these children's more limited general processing capacity and the nature of the task.

Communication between caregivers and children with moderate to severe motor impairments is a tremendous challenge, and one that deserves attention as a central component of early intervention programs. This article examines a caregiver-training program that explored key elements to creating strong communicative interactions between young children with moderate to severe motor impairments and their primary caregiver. Three caregiver-child dyads participated in a 3-week treatment program teaching caregivers how to provide communicative opportunities, wait for a clear communication signal from their children, recognize their children's signal, and finally, shape a more advanced communicative behavior. These adult behaviors were designed to increase the children's use of conventional engaging signals of communication. Results revealed that caregivers demonstrated success learning all behaviors except for shaping during the brief treatment period. Children's engaging communicative behaviors increased correspondingly with the caregivers' changes. These findings have positive implications for caregiver training. Implications for "best practice" are considered.

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Infantile spasms respond to ACTH, and levels of the hormone in cerebrospinal fluid of untreated infants with this disorder were found to be lower than in age-matched controls. In this study we analyzed cerebrospinal fluid Cortisol and ACTH using improved immunoassays in a larger cohort of infants with infantile spasms. Analysis of 20 patients and 15 age-matched controls revealed significantly lower levels of both ACTH and Cortisol in the cerebrospinal fluid. These data, combined with the efficacy of ACTH and glucocorticoids for infantile spasms, support an involvement of the brain-adrenal-axis in this disorder.