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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.