Strength measurements in patients with Dravet Syndrome.

BACKGROUND: Dravet Syndrome (DS) is a developmental and epileptic encephalopathy, characterized by drug resistant infantile onset seizures and cognitive and motor impairment. Walking problems progressively occur and crouch gait is frequently observed. Muscle weakness is hypothesized as contributing impairment. Yet, so far, no studies have performed strength measurements in patients with DS, most likely due to cognitive impairment. AIMS: To determine the feasibility and validity of strength measurements in the framework of gait analysis and to outline strength problems in patients with DS. METHODS: Manual muscle testing, dynamometry (hand grip strength and handheld dynamometry) and functional tests (underarm throwing, standing long jump, sit-to-stand, stair climbing) were performed in 46 patients with DS. Results were compared to age-related reference values from literature. RESULTS: Forty one percent (19/46) of the patients (aged 5.2-24.8 years, median: 15.8 years) accomplished all measurements and scored generally below the fifth percentile of norm values. The remaining 59% (27/46) was not able to complete all strength assessment due to cognitive, behavioural and motor difficulties. Handheld dynamometry seemed most sensitive and specific to detect isolated muscle strength. Validity of the functional tests was controversial, as motor proficiency, balance and coordination may interfere. CONCLUSION: Although measuring strength in patients with DS was challenging in the context of gait analysis, decreased muscle strength was observed in patients that could perform strength measurements. Handheld dynamometry is preferred over functional tests for future investigations of muscle strength and its interference with gait are required for better understanding of walking problems.