Motor Neurone Disease (MND) is the name given to a group of diseases in which the nerve cells (neurones) that control muscles undergo degeneration and die. Amyotrophic Lateral Sclerosis (ALS), Progressive Muscular Atrophy (PMA), Progressive Bulbar Palsy (PBP) and Primary Lateral Sclerosis (PLS) are all subtypes of motor neurone disease.
MND is the widely used generic term in the United Kingdom, Australia and parts of Europe, and ALS is used more generically in the United States, Canada and South America.
These diseases are also sometimes known as Maladie de Charcot and are often referred to in the United States as Lou Gehrig’s Disease, after the famous baseball player who died of the disease.
By any name, ALS/MND is characterised by progressive degeneration of the motor nerve cells in the brain and spinal cord. The motor cells (neurones) control the muscles that enable us to move around, speak, breathe, and swallow. With no nerves to activate them, muscles gradually weaken and waste. Symptoms may include muscle weakness and paralysis, as well as impaired speaking, swallowing, and breathing. Progress is generally rapid, with an average life expectancy of between 2 and 5 years from the onset of symptoms.
Though it can affect anyone, ALS/MND is more often found in the 40 to 70 year age group.
The impact on the community of ALS/MND is usually measured by the incidence and prevalence of the disease. Incidence is the number of new cases added in a defined period, usually a year. Prevalence is the number of cases existing at any point in time. The incidence of ALS/MND is 2 per 100,000 of total population, while the prevalence is around 6 per 100,000 of total population. Research has found that the incidence is higher in people aged over 50 years. A small proportion of cases (~10%) are familial (inherited) while the majority of cases (~90%) are still considered sporadic or singleton. However, research is continuing to further reveal genetic associations with ALS/MND. This will likely mean that many cases considered sporadic may also have a genetic contribution. Further research is needed to truly establish the extent of the genetic contribution to causing and/or moderating ALS/MND. The Alliance believes that all people diagnosed with ALS/MND should have genetic counselling and testing if they chose to.
Although classified as a rare disease based on its prevalence, ALS/MND in fact quite common. There are approximately 140,000 new cases diagnosed worldwide each year. That is 384 new cases every day!
The disease affects each individual differently and can have a devastating impact on family, carers, and friends. The rapidly progressive nature of the disease requires constant adaptation to increasing and changing levels of disability, which in turn require increased levels of support.