Gait deviations in patients with dravet syndrome: A systematic review

Background: Dravet Syndrome is a rare developmental and epileptic encephalopathy characterised by epileptic seizures, cognitive impairment and motor disorders. Gait is markedly impaired and could benefit from targeted intervention to improve quality of life for patient and caregivers. Objective: To establish the state of the art regarding gait deviations in patients with Dravet Syndrome. Methods: A systematic search was performed in Pubmed, Web of Science, Science Direct and Embase. Studies that assessed gait deviations in patients diagnosed with Dravet Syndrome using clinical observation, video gait analysis or three dimensional (3D) gait analysis and reported gait characteristics, spatiotemporal or kinematic outcomes were included. Screening, quality assessment and data extraction were performed by independent reviewers. Results: Out of a total of 478 citations, nine articles were included. The total study population had an age range from 2.5 to 47 years. Three studies used clinical observation, three studies video analysis and three studies 3D gait analysis. Crouch gait was observed in about half of the population next to a variety of other gait deviations such as parkinsonian and cerebellar gait. Other findings included abnormalities in spatiotemporal parameters and kinematics, passive knee extension deficits, skeletal malalignment and neurological signs. Conclusions: A variety of gait characteristics was observed with crouch gait being the most reported gait pattern. Inconsistency in methods and findings from clinical and instrumented evaluation impede thorough understanding of the causal mechanism and evolution behind these deviations. (C) 2019 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.