Миеломенингоцеле при расщеплении позвоночника: нейропсихологические последствия, связанные с работой мозга^*

В данной статье рассматриваются физические, неврологические и когнитивные фенотипы миеломенингоцеле при расщеплении позвоночника (spina bifida myelomeningocele, МРП) – не летального, но наиболее распространенного врожденного дефекта нервной трубки, поражающего центральную нервную систему. После рассмотрения физических и неврологических фенотипов авторы объясняют, каким образом изменение данных параметров принципиально влияет на когнитивный фенотип МРП. Когнитивный фенотип представляет собой типичный когнитивный профиль с повышенными показателями ассоциативного обучения на основе правил и сниженными показателями в комплексных интегративных процессах. Данный фенотип связан с ядерными дефицитами восприятия времени, внимания и моторных навыков, которые возникают на ранней стадии развития из-за пороков развития мозга, затрагивающих мозжечок, средний мозг и мозолистое тело. Вариабельность клинических исходов миеломенингоцеле при расщеплении позвоночника также связана с уровнем поражения спинного мозга, вторичными эффектами гидроцефалии и ее лечением, а также с психосоциальным средовым фактором. Раннее вмешательство и комплексный подход, основанный на понимании когнитивного профиля, типичного когнитивного фенотипа и его вариаций, являются важными факторами в контексте помощи людям с миеломенингоцеле при расщеплении позвоночника, направленной на максимально возможное улучшение когнитивного развития, адаптивных функций и качества жизни.

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