Abstract

Abstract Impairment in the activities of daily living (ADL) in motor neuron disease (MND) has been little investigated. The contributions of both behavioural and motor changes on functional performance have not been explored. A postal survey in New South Wales, Australia, included assessments of ADL, behavioural change (carer-based) and MND severity. Eighty-two patients were subdivided into groups according to onset presentation: bulbar (n=23) and limb (n=59). There were significant differences in ADL performance between limb and bulbar onset groups depending on ADL task. Disability was also dependent on disease severity as measured by the Amyotrophic Lateral Sclerosis Functional Rating Scale - Revised (ALSFRS - R) score. Importantly, variance in ADL scores was dependent on both motor and behavioural factors. This study confirms the progressive disabling nature of MND, which is dependent on disease severity and shows qualitative differences depending on onset presentation. A model combining motor and behavioural changes explained 57% of variance on ADL performance, with important implications for clinical intervention.