Amyotrophic Lateral Sclerosis

Amyotrophic Lateral Sclerosis

amyotrophic lateral sclerosis This is a  motor neuron type of disorder caused by degeneration of the motor neurons in the ventricular horn of the spinal cord and cortical neurons that provide their efferent input.  That is  this defect is mainly affecting  the motor neurons that reach out from the brain towards the spinal cord and towards the muscle.

This is often called Lou Gehrig’s disease after the famed baseball player whose struggle with this disease and death in 1941 brought it national attention.

For reasons that are not clearly known, the nerve cells of the brain and spinal cord that control voluntary muscle movement gradually deteriorate. As a result, muscles waste away, leading to paralysis and death, usually in two to five years.

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The lower motor neuron , which control a wide range of things like movement of the  limbs, swallowing, and even some aspects of breathing  are usually getting affected. There will be weakness and atrophy of the muscle, fasciculations, spasticity, dysarhtria, dysphagia  and respiratory compromise.

The Autonomic  and sensory nervous, affecting  functions like sweating  and thinking are usually spared, but may be involved in some patients.

It almost always strikes after the age of 40, and it affects more men than women.

Probable Causes

Genetic Inherent – 5-10 % diseases are seen in a family tree itself.  The defective gene is believed to be preventing the body from producing  the enzyme superoxide dismutase. Usually this enzyme helps to neutralize the free radicals which cause high damages to the body.

Environmental Toxins- May be a reason for non inherited ALS as the investigation shows the enzyme damage.

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Other Causes- Some believe that the heavy metal poisoning, pesticide poisoning, some viral infections, physical trauma etc. are usual contributors of ALS.

Excitotoxicity- Due to over stimulation of the nerve cells, that controls movements, by the neurotransmitter glutamate leads to eventual death of the cell