Motor Neurone Disease Pathophysiology

NewsGuard 100/100 Score

Motor neurone disease is a progressive degenerative disorder affecting specialist nerve cells called motor neurons that control muscle movement.

Motor neurons are vital in controlling the various activities of the body that involve muscle movement including walking, running, speaking, swallowing, breathing and gripping. As the disease progresses, these actions become difficult and eventually the person is unable to carry  them out at all.

The skeletal muscles are muscles that are voluntarily controlled such as those in the legs used for walking. There muscles are innervated by a group of nerves called the lower motor neurones. The lower motor neurons are found in the ventral horn of the spinal cord and are responsible for all voluntary movements. The lower motor neurons are activated by the release of glutamate form the upper motor neurones in the brain stem.

In motor neurone disease, damage to the ventral horns of the spinal cord leads to atrophy of the ventral roots. Neurons in the frontal and temporal lobes also degenerate. Studies looking at the nerve damage caused by motor neuron disease have revealed the presence of sponge-like deformities called spongiosis, skein like inclusions and bunina bodies as well as the formation of vacuoles.

In around 5% of people who suffer from motor neurone disease, defective genes are thought to play a role in contributing to the disease. A number of genes have been identified that cause the inherited form of this disease and these include: SOD1, TDP-43, FUS,VCP, C90RF72, SQSTM1 and Profilin1.

Further Reading

Last Updated: Jul 7, 2023

Dr. Ananya Mandal

Written by

Dr. Ananya Mandal

Dr. Ananya Mandal is a doctor by profession, lecturer by vocation and a medical writer by passion. She specialized in Clinical Pharmacology after her bachelor's (MBBS). For her, health communication is not just writing complicated reviews for professionals but making medical knowledge understandable and available to the general public as well.


Please use one of the following formats to cite this article in your essay, paper or report:

  • APA

    Mandal, Ananya. (2023, July 07). Motor Neurone Disease Pathophysiology. News-Medical. Retrieved on March 04, 2024 from

  • MLA

    Mandal, Ananya. "Motor Neurone Disease Pathophysiology". News-Medical. 04 March 2024. <>.

  • Chicago

    Mandal, Ananya. "Motor Neurone Disease Pathophysiology". News-Medical. (accessed March 04, 2024).

  • Harvard

    Mandal, Ananya. 2023. Motor Neurone Disease Pathophysiology. News-Medical, viewed 04 March 2024,


The opinions expressed here are the views of the writer and do not necessarily reflect the views and opinions of News Medical.
Post a new comment