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.

Strengths and limitations of this study

• This population-based study used multiple clinical and health-service data sources across Western New South Wales Local Health District, with records cross-referenced to maximise case ascertainment and remove duplicates.

• The 2023-2025 study period provides a pre-notification epidemiological baseline preceding the introduction of mandatory motor neuron disease (MND) notification in New South Wales in September 2026.

• Epidemiological and geographical analyses used standardised population denominators and established Australian classifications of remoteness and land use.

• The relatively small number of people with MND limited statistical precision, particularly for subgroup and Local Government Area-level analyses.

• Individual-level genetic, occupational and environmental exposure data were unavailable, limiting investigation of factors potentially underlying demographic and geographic variation.