Motor neurone disease deaths are rising. We need more data to find out why

Motor neurone disease (MND) may be classified as a rare disease (one that affects fewer than 1 in 2000 people), but prevalence is a deeply flawed measure for a disease that kills quickly. MND has a median survival of less than two years from diagnosis and people do not live long enough to accumulate in the statistics. Counting how many patients are alive at any one time dramatically underestimates the true impact of MND; mortality data tell the real story.

Our research, published in the Medical Journal of Australia, reveals that MND killed 781 Australians in 2023, nearly two-thirds the number (1266) who died in motor vehicle accidents that year. Yet MND attracts only a fraction of the political attention, public investment and urgency accorded to the road toll. Even if it is considered (arbitrarily) a rare disease, shouldn’t our governments pay greater attention to a terminal illness that now accounts for one in every 234 deaths? 

Perhaps even more alarming than the total number of deaths from MND is the trend our analysis of Australian Bureau of Statistics mortality data identified: MND-related deaths more than tripled over the past 37 years and we can’t avoid asking why. Population ageing, improved diagnosis and greater awareness may contribute in a minor way but they don’t explain a three-fold increase in a single generation. The uncomfortable truth is that the rise cannot yet be explained.

Environmental factors are at play

Only 10% of MND cases are genetic, inherited within a family in autosomal dominant fashion. The remaining 90% of cases have no family history, meaning the cause must lie outside our genes. When prevalence of a sporadic disease increases on this scale, it suggests a direct association with the environment and geographic variance adds weight to that hypothesis.

MND mortality rates are not uniform across Australia: people living in regional areas are 40% more likely to die from MND than those in major cities, and mortality is dramatically higher in Tasmania (by 40%) and South Australia (by 20%) than in New South Wales. These patterns have long been observed by regional neurologists but not previously confirmed through national data.

When risk follows geography, identifying exposure is critical. What differs between regional and urban Australia? Agriculture, pesticides, industrial chemicals and other occupational exposures are potential suspects, but we can’t begin to test causal links because Australia doesn’t count MND patients systematically.

MND is not yet a notifiable disease: we rely on fragmented registries, voluntary reporting, and delayed death certificates. Without complete case capture, we cannot identify clusters, exposures, or causal mechanisms. Without understanding mechanisms, therapies are necessarily generic and empiric, and without mechanistic therapies, cure is impossible and MND will remain universally fatal.

We must make MND a notifiable disease, and surveillance is the first step. In New South Wales, we have begun a comprehensive surveillance initiative in the Riverina region; this must be urgently expanded statewide and nationally, with proper resourcing. But surveillance alone is not enough.

Early diagnosis and access to multidisciplinary care 

Only 4% of Australia’s neurologists work outside major metropolitan regions as their primary place of practice. Our general practitioner colleagues handle MND at the front line, but they are loaded with a vast spectrum of disease presentations, symptoms and health-related issues, and it can be challenging for GPs to identify a disease they have not been exposed to regularly.

GPs need tools to support complex decision-making and identify specialised conditions, such as our “red flag” ThinkMND online algorithm. When a proper MND diagnosis is missed or delayed, patients often undergo unnecessary ENT procedures for slurred speech (bulbar onset MND) or orthopaedic or neurosurgical procedures for weak limbs (limb onset MND).

MND can devastate lives long before it ends them. Patients can lose the ability to walk, speak, swallow and breathe, often within months. Families become full-time carers, watching rapid deterioration unfold in real time. The emotional, financial, and physical burden is enormous, and our government needs to pay attention to this: in 2025, economic modelling by MND Australia estimated the real cost of disease associated with MND as $5 billion.

Access to expert multidisciplinary care — including neurology, respiratory support, physiotherapy, speech pathology, nutrition, genetic counselling and social work — is proven to extend survival and improve quality of life. Yet outside major cities, access for MND patients is patchy or non-existent. Patients are forced to travel long distances, delay care, or go without.

We must declare MND a notifiable disease and build a comprehensive national database. More importantly, we must implement regional multidisciplinary hubs, so care comes to patients and not the other way around. We need to provide equipment, support, and dignity to families living with this terminal diagnosis.

Australian healthcare can do better. Calling MND “rare” is not just inaccurate but a dangerous excuse for inaction and apathy; a disease killing 800 Australians a year and rising fast, that’s unevenly distributed and largely unexplained should command our complete attention.

Dr Carol Lee is a Project Lead for Motor Neurone Disease Research at the MND Research Centre, Macquarie University

Professor Dominic Rowe AM is Professor of Neurology in the Macquarie Medical School, Macquarie University