Motor neuron disease is a group of brain diseases that affect the nerve cells controlling your muscles. Amyotrophic lateral sclerosis (ALS) is the most common form of motor neuron disease, and it’s the same disease that Stephen Hawking has (although he appears to have a very rare form which hasn’t affected his lifespan). In America it’s also known as Lou Gehrig’s disease, because of its association with a famous baseball player. It’s a progressive disorder, which means it will get worse over time, and depending on the type of motor neuron disease it can affect different parts of your body. There is no cure currently, but many of my colleagues are currently researching methods into treating motor neuron disease.
Motor Neurone Disease (MND) is a disease of the motor neurones! i.e. the cells that control our voluntary muscles (e.g. speaking, walking, breathing, swallowing). There are types though (these will look like strange scientific names, but bear with me!): Amyotrophic lateral sclerosis (ALS – what Stephen Hawking has!), primary lateral sclerosis, progressive muscular atrophy, progressive bulbar palsy and pseudobulbar palsy.
Normally, messages from nerve cells in the brain (upper motor neurons) send messages to nerve cells in the brain stem or spinal cord (lower motor neurons). The upper motor neurons tell the lower motor neurons to produce movements like walking or chewing. When there are disruptions in the signals, the muscles can no longer work properly and they begin to waste away. They can also twitch uncontrollably and eventually the signals stop all together.
The diseases can affect children or adults. In children, it is usually genetic. One of the genes identified is called ALS2, which codes for a protein that is in motor neurons. If this protein is defective, then the neurons don’t work properly.
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