Facts about ALS

What is ALS?
ALS or Amyotrophic lateral sclerosis is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord which causes muscle weakness, paralysis and in severe situation, respiratory failure. 

A-myo-trophic is a a Greek language where 'A' means negative or no, 'myo' refers to muscle and 'trophic' which means nourishment.  Simply put, 'amyotrophic' means 'no muscle noursihment'.  And when a muscle has no nourishment, it 'atrophies' or it wastes away, meaning it becomes smaller.  'Lateral' is the area in the spinal cord where part of the nerve cells that signals and controls the muscle is located.  When this area deteriorate, it leads to 'sclerosis', the scarring or hardening in the region.

ALS is also called  motor neurone disease (MND), Charcot disease, and Lou Gehrig's disease.  Lou Gehrig is a baseball legend who had ALS and whom it is named after.

How does ALS affect the body?
When motor neurons degenerates, the body loses the ability to initiate and control all the voluntary movement.  The neurons can no longer send impulses to the muscle fibers that normally result in movement.  And when the muscle no longer receive messages from the motor neurons, it will then shrink.  These dead neurons will make the brain lose its ability to initiate and control muscle movement that could lead to total paralysis.

What are the symptoms of ALS?
Early symptoms of ALS are typically ovbious weakness and/or muscle atrophy. But it depends on which motor neurons in the body are damaged first.  75% of the people who had the disorder experience 'limb onset' – symptoms in the legs (like awkwardness when walking or running, tripping or stumbling) and arms (difficulty in tasks like butoning a shirt, writing or even turning a key in the lock).  The remaining 25% experience 'bulbar onset' which includes difficulty in speaking clearly, swallowing, loss  of tongue mobility.  But there are also those who experienced 'respiratory onset' wherein the muscles that support breathing are affected.  The senses, including hearing, sight, smell, taste and touch, are not affected by ALS.

What are the causes of ALS?
Although the cause of ALS is not yet completely known or understood, there are scientific researches that have found out ALS may have some genetic links.  Scientists have discovered that the most common ALS-causing mutation is SOD1 gene.  A 2011 research found a genetic abnormality known as hexanucleotide in the C9orf72 gene which is present in both ALS and frontotemporal  dementia patients and that these cases may be related.

Who can have ALS?
ALS is said to be the most common of the five motor neuron diseases.  Since this disease has no clear factor or cause, it can occur randomly.  It is said to be most common among white males, non-Hispanics and those in the 60-69 age bracket.  But younger and older people alike can develop this disease.  Most cases of ALS are inherited.

How can ALS be treated?
No cure has been found yet for ALS.  But only one drug is approved for usage, the riluzole.  This drug, though, does not repair the already-damaged motor neurons.  It only prolong the survival of the patient for several months and not totally curing it.


 Published at: 09/01/2014