Deconstructing Dravet syndrome neurocognitive development: A scoping review

Dravet syndrome (DS) is a rare severe epilepsy associated with slowed psychomotor development and behavioral disorders from the second year onward in previously seemingly normal child. Among cognitive impairments, visuospatial, sensorimotor integration, expressive language deficits are consistently reported. There have been independent hypotheses to deconstruct typical DS (dorsal stream vulnerability, cerebellar-like pattern, integration deficit), but an encompassing framework still lacking. We performed scoping review of existing evidence map current understanding developmental profiles summarize on suggested frameworks. searched PubMed, Scopus, PsycInfo, MEDLINE identify reports focusing and/or abnormalities published between 1978 March 15, 2020. followed Preferred Reporting Items for Systematic reviews Meta-Analyses extension Scoping Reviews (PRISMA-ScR) guidelines. Twenty-one were selected tabulated by three reviewers based predefined data extraction eligibility forms. Eighteen provided assessments global intelligence quotients variable degrees impairment. Eleven analyzed single subitems contribution scores: these showed larger impairment performance scales compared verbal ones. Studies assessing specific functions demonstrated deterioration early visual processing, fine gross motor abilities, visuomotor auditory-motor spatial visuo-attentive executive functions, language. Behavioral abnormalities, reported 14 studies, highlighted autistic-like traits attention hyperactivity disorders, slightly improving age. The profile some may be enclosed within unified theoretical main advanced: pervasive deficit, occipito-parietofrontal circuit stream) dysfunction coexistent cerebellar deficit.