Neurological presentations and cognitive outcome in Sturge-Weber syndrome

CONCLUSIONS: This is the largest reported series documenting detailed developmental profiles of children with SWS, including ID and ASD. PWS(+) shows high rates of ID and language disorder. PWS(-) SWS has a more favourable outcome. Cognitive outcome is contingent on number of affected lobes and bilateral involvement. Epilepsy exerts an additional deleterious effect on language and cognition. A high percentage of children have a history of status epilepticus, with evidence that this impacts language and cognitive outcomes. Acutely acquired neurological deficits did not penalise either. Regular structured clinical and developmental assessment permit greater identification of neurological and neurodevelopmental impairments in SWS, and appropriate support.PMID:34293629 | DOI:10.1016/j.ejpn.2021.07.005
Source: European Journal of Paediatric Neurology - Category: Neurology Authors: Source Type: research