Extrapyramidal deficits in ALS: a combined biomechanical and neuroimaging study

摘要: Extrapyramidal deficits are poorly characterised in amyotrophic lateral sclerosis (ALS) despite their contribution to functional disability, increased fall risk and quality-of-life implications. Given the concomitant pyramidal cerebellar degeneration ALS, clinical assessment of extrapyramidal features is particularly challenging. The comprehensive characterisation of postural instability ALS using standardised assessments, gait analyses computational neuroimaging tools a prospective study design. Parameters initiation anticipatory adjustment phase (APA) execution (EP) were evaluated patients with without healthy controls. Clinical analysis parameters interpreted context brain imaging findings. exhibit impaired an altered APA phase, poor dynamic control significantly decreased braking index. Consistent profile, “unsteady” have reduced caudate stem volumes compared “steady” patients. Our findings highlight that rating scale (ALSFRS-r) does not account for deficits, which major contributors impairment subset Basal ganglia only contribute cognitive behavioural but also adds heterogeneity motor disability.