Motor neuron disease in rural Australia: a population-based observational study of epidemiology, clinical characteristics and regional variation

Objectives: To characterise the epidemiology, clinical features, and geographic distribution of motor neuron disease (MND) within a rural Australian population. Design: Retrospective, population-based epidemiology study. Setting: Western New South Wales Local Health District (WNSWLHD), Australia, from 1 January 2023 to 31 December 2025. Participants: Fifty-one individuals diagnosed with MND identified through Central West Neurology and Neurosurgery, Orange Palliative Care Service, and the WNSWLHD Neurodegenerative Community Care Team. Main outcome measures: Crude and age-standardised incidence, prevalence and mortality rates of MND; demographic and clinical characteristics; geographic distribution of prevalence. Results: The all-age crude incidence of MND was 4.01 per 100,000 person-years (95% CI, 2.78-5.60), prevalence was 8.14 per 100,000 population (95% CI, 6.33-10.30), and mortality was 3.77 per 100,000 person-years (95% CI, 2.58-5.33). Females comprised 51% of patients and showed higher incidence, prevalence and mortality than males. Bulbar-onset MND accounted for 47% of patients, including 61% of females. MND prevalence varied across the health district, with the highest burden observed in central and south-eastern regional areas. Patients travelled on average 139 km to access neurological care, with a diagnostic delay of nine months. Conclusions: MND incidence and mortality are high in this rural Australian population compared with national and global estimates, with an overrepresentation of bulbar-onset disease and altered sex distribution. These findings provide an important baseline for population-level MND surveillance, following the introduction of mandatory notification in NSW, Australia. Findings also highlight the need for strengthened rural healthcare services, and further research of potential genetic and environmental contributors to MND risk.