Learning About: Motor Neuron Disease

Disclaimer: This is a collection of information based on my own research, please do not use this as a direct source of medical advise/information as I am not educated enough to provide that sort of help! I found out about this disorder and decided to research it further, all sources are linked at the end of the article.

What is motor neuron disease?

Motor Neuron Disease (MND) is an umbrella term for a range of neurological disorders where motor neurones are destroyed/die prematurely. 

Motor neurons are a type of nerve cell that transmit messages from the central nervous system (the brain and spinal cord) to effectors. Effectors are cells that respond to a stimulus, for example muscle cells.  

Considering that motor neurones are affected, movement is greatly affected by MND. Since muscles don't receive the messages to move from the brain, increasingly less movement occurs causing muscles to weaken overtime. The effect of this can be felt greatly as the disease progresses as the inability to grip an item can turn into the inability to walk or move. MND can also lead to a condition called respiratory inefficiency. 

How does MND occur?

The process of the motor neurones mentioned above dying is called neurodegeneration. Neurodegeneration is also common in conditions such as Parkinson's and Alzheimer's disease however in these diseases the death of neurones occur in the brain. 

Inherited MND is caused by a gene mutation that is inherited from one or both parents, depending on the disease being recessive or dominant.

Possible Other Ideas for Causes:

  1. Mutations in RNA genes might result misfolded proteins that form aggregates. These are abnormal clumps of protein that can be found inside of the neurones which leads to neurodegeneration. RNA is used by the cell to figure out how to order amino acids when connecting them together to form proteins so the wrong order of amino acids being assembled can result in incorrect folding.
  2. The transport systems used in cells to get rid of waste from activities like respiration can become disrupted. This causes waste to build up in the cell resulting in neurodegeneration.
  3. Faulty Glial cells mean that motor neurones can't receive the nutrients they need to function properly. Glial cells are cells that provides physical and chemical support to neurons.
  4. Glutamate, a neurotransmitter, triggers a lot of activity in nerve cells which might result in damage to the cells. Neurotransmitters are chemicals that nerve cells release to pass messages to other cells.
  5. The Mitochondria in motor neurones might not function properly meaning the cell doesn't have enough energy to function normally.

Stephen Hawking

Stephen Hawking had a type of MND called Amyotrophic Lateral Sclerosis (Amyotrophic = muscles waste away/have no nourishment, lateral=to the side, sclerosis = hardening of tissue). ALS affects the upper and lower motor neurones. Upper motor neurones are motor neurones in the CNS which pass messages to the lower motor neurones which branch away from the spinal cord to the effectors.

ALS meant that Hawking progressively lost control over most of his muscles which restricted him to using a wheelchair and a voice synthesizer to go about his everyday life. However to say that this prevented him from living a fulfilling and inspiring life would be far from the truth. His work surrounding science and the universe contributed to the field greatly and inspired many.

Hopefully technology continues to progress so that the kinds of aids Hawking used improve and become more accessible to those who can utilise them. Science runs off of curiosity, but medicine runs off of the compassion we have for the human condition, so here's to innovation!

Sources:

https://www.ninds.nih.gov/health-information/disorders/motor-neuron-diseases
https://www.brainresearchuk.org.uk/neurological-conditions/motor-neurone-disease
https://www.nhsinform.scot/illnesses-and-conditions/brain-nerves-and-spinal-cord/motor-neurone-disease-mnd#:~:text=MND%20happens%20when%20specialist%20nerve,gripping

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