ALS stands for Amyotrophic Lateral Sclerosis. ALS is also called motor neuron disease (MND). This is a disease that causes the death of neurons that control the voluntary muscles. These muscles are the group of muscles that we can control as we want them to. They are also called skeletal muscles that are attached to the bones and are responsible for locomotion. Typical features of ALS are muscle twitching, stiffening of muscles, and a gradual deterioration in the muscle size leading to weakness in the body.
Characteristics of ALS:
The condition ALS begins with typical weakness in the body which gradually develops into stiffening of muscles and joints and the patient starts developing difficulty in speaking as well as swallowing. Many of the patients also start suffering from difficulty in thinking and few undergo behavioral changes. Patients also suffer from pain as well. The condition further progresses eventually into the restricted movement of hands, legs, and difficulty in breathing. The patient also is unable to walk or help himself in any way.
In around 95 % of cases, the cause is unknown but researchers believe that the causes include the environmental factors as well as genetic factors. The remaining 5% of the cases are to be believed as hereditary wherein the patient’s family history shows that someone had suffered from a similar disorder. Usually, the messages are transmitted from the motor neurons in the brain which are called as the upper motor neurons to the motor neurons of the spinal cord and motor nuclei of the brain which are known as lower motor neurons.
ALS is a condition that involves damage of both upper and lower motor neurons which gradually degenerate and die and generally, diagnosis and prognosis depend upon presenting signs and symptoms of the patient. Henceforth tests are conducted to rule out probable causes.
When it comes to the treatment aspect there is no known cure available for ALS. Generally, in case of any neuron damage and disorder, the main treatment protocol would be focussing on improving the symptoms of the patients.
The medication riluzole helps in extending the life of the patient by 3 to 4 months. Also, mechanical ventilation is used to ease the symptoms in the patients but it cannot stop the disease from progressing. ALS can occur at any age but most of the time the disease is seen affecting people above 55 yrs of age. Usually, in most cases, the patients die due to the respiratory failure usually within the first 6 yrs of the appearance of the first symptom in the body. Among 8% to 10 % of the patients happen to survive for 10 years or more.
The Early History of ALS:
The disease ALS was first found in 1824 by Charles Bell. Later in the year, 1869 Jean-Martin Charcot developed the connection between the symptoms and the underlying neurological problems. Thus in 1874 Amyotrophic Lateral Sclerosis term was coined by Jean-Martin Charcot. This disease is often termed as Lou Gehrig’s disorder also when in the year 1939 it affected the famous baseball player Lou Gehrig. Thus in the 20th century, the disease was well known in the United States.
Motor neuron disease is a bunch of neurological disorders that affect the motor neurons of the body affecting the voluntary muscles of the body. These are:
Amyotrophic Lateral Sclerosis( ALS )
Primary Lateral Sclerosis( PLS )
Progressive Muscular Atrophy( PMA )
Progressive Bulbar Palsy( PBP )
Monomelic Amyotrophy( MMA )
ALS is further identified as a service-connected disease by the UN Department of Veteran’s Affairs. Further classified into 2 types which are:
Sporadic ALS: This affects around 80% of the people. This means the disease affects at random without any family history. Although family members of the people are at risk of developing this disease, the risk is low and most will not necessarily develop ALS.
Familial ALS: Around 10% to 15% of the people come in this category wherein an individual does have a family history or genetic background of this disease. At least one parent needs to carry the gene responsible for the familial type of ALS. Mutations linked to a dozen of genes might be a responsible cause for familial ALS. Researches have also associated the same genes as the cause of frontal-temporal lobe dementia.
1. What causes ALS?
Ans: This is one among the motor neuron disorders which causes gradual degeneration and death of motor neurons affecting the locomotion of the body. There is no particular cause for the disease to occur, the cause can be random or hereditary if any parent has this gene and passes on to the children.
2. Who are the Famous Persons who had ALS?
Ans: The first person in whom this disease was recognized was Lou Gehrig who was a famous baseball player. Stephen Hawking is said to be the longest ALS survivor.