Amyotrophic Lateral Sclerosis (ALS)

What is ALS (amyotrophic lateral sclerosis) or MND (motor neuron disease)?

ALS (amyotrophic lateral sclerosis), pronounced “a-my-o-TROE-fik LAT-ur-ul skluh-ROE-sis”, is also known as motor neuron disease (MND), although it is actually a type of motor neuron disease. Some refer to ALS as Lou Gehrig’s disease after a famous American baseball player, Lou Gehrig, was the first to be diagnosed with the condition in the 1930s.

ALS is a progressive and fatal disease affecting the central nervous system and brain. It destroys the nerve cells in the body which leads to disability and paralysis in the sufferer during the late stages of disease development.

Motor neurons are nerve cells that are found in the spinal cord and central nervous system. Each neuron contains a specialised fibre that projects, or rather, communicates with other organs outside of the spinal cord in order to indirectly or directly control muscles and glands. These neurons conduct impulses from the central nervous system to glands, muscles or effector tissues, transmitting signals from the spinal cord to the muscles which aid in coordination and movement, telling the systems in the body how to function.

In ALS, neurons are slowly destroyed. The slow deterioration of the neurons is referred to as neurodegeneration.

Motor neurons control vital muscle activities such as:

• Walking
• Gripping
• Speaking
• Breathing
• Swallowing

As ALS progresses and destroys motor neurons, the brain is no longer able to effectively give commands to the body, and these functions become increasingly difficult, eventually becoming impossible to perform over time. This is why those with late stage ALS usually become paralysed.

It is not yet clear what the exact cause of neurodegeneration is and as such, ALS does not have a cure. ALS is referred to as a motor neuron disease that is familial. Roughly 5% of patients with the condition have a family history of the disease or a family member with a condition known as frontotemporal dementia.

Frontotemporal dementia, also known as FTD is a progressive disease that results in damage to the frontal lobes of the brain. The typical symptoms are issues with speech, compulsive behaviour, memory issues and mood changes.

In the majority of inherited cases, faulty genes have previously been identified as being a significant contributing factor to the condition developing.

There is no single diagnostic test or procedure that can be used to accurately diagnose ALS. Typically, a nervous system and brain specialist known as a neurologist will conduct a series of tests during the diagnostic process.