SUMMARYThe diagnosis, evaluation, and management of infantile spasms (IS) continue to pose significant challenges to the treating physician. Although an evidence-based practice guideline with full literature review was published in 2004, diversity in IS evaluation and treatment remains and highlights the need for further consensus to optimize outcomes in IS. For this purpose, a working group committed to the diagnosis, treatment, and establishment of a continuum of care for patients with IS and their families-the Infantile Spasms Working Group (ISWG)-was convened. The ISWG participated in a workshop for which the key objectives were to review the state of our understanding of IS, assess the scientific evidence regarding efficacy of currently available therapeutic options, and arrive at a consensus on protocols for diagnostic workup and management of IS that can serve as a guide to help specialists and general pediatricians optimally manage infants with IS. The overall goal of the workshop was to improve IS outcomes by assisting treating physicians with early recognition and diagnosis of IS, initiation of short duration therapy with a first-line treatment, timely electroencephalography (EEG) evaluation of treatment to evaluate effectiveness, and, if indicated, prompt treatment modification. Differences of opinion among ISWG members occurred in areas where data were lacking; however, this article represents a consensus of the U.S. approach to the diagnostic evaluation and treatment of IS.

By several accounts, reading single words may be accomplished either by sequentially transcribing orthographic units into their corresponding sounds (an indirect route), or by directly associating a visual word form to the semantic or articulatory representation (a direct route). By contrast, the similar task of naming objects must rely only on a direct route, since objects cannot be "sounded out." To study the localization of cognitive processes specific to reading, we used positron emission tomography (PET) to measure regional cerebral blood flow while subjects named words and pictures of objects silently or aloud. Group averages of blood flow changes were obtained for experimental vs. control tasks. Object and word presentations elicited similar blood flow increases in extra-striate visual cortices compared with a visual noise control. Silent reading invoked a neural network very similar to that seen when subjects named objects silently, consistent with a "direct" route. Naming objects aloud produced the addition of motor output regions to this network. By contrast, oral reading produced a markedly different pattern of activated regions, suggesting reliance on a separate phonological pathway. These results provide support for the dual coding hypothesis in reading and challenge the use of strict hierarchical models of cognitive operations in PET activation studies.

Early seizure onset and atypical handedness, as well as the location and nature of pathologic substrate, are important factors in language reorganization.

A panel of fMRI language paradigms may be more accurate for evaluating partial epilepsy patients than a single task. A panel of tasks reduces the likelihood of nondiagnostic findings, improves interrater reliability, and helps confirm language laterality.