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Amyotrophic Lateral Sclerosis (ALS)


Amyotrophic lateral sclerosis (ALS) is also known as Lou Gehrig's Disease. It is neurodegenerative disease that affects nerve cells in the brain and the spinal cord. The motor neurons travel from the brain to the spinal cord and from the spinal cord to the muscles in the body. The progressive deterioration of the motor neurons in ALS finally lead to their death. When the motor neurons die  then the ability of the brain to initiate and control muscle movement is lost.

ALS affects 1 out of every 100,000 people. ALS most commonly strikes people between 40 and 60 years of age but younger and older people also develop the disease. Men are affected more often than women.

Types of ALS

There are three types of ALS classic sporadic, familial, and the Mariana Island forms. These are as follows

  • Classic sporadic - This form is sporadic in that it cannot be traced to ancestors suffering with the illness.
  • Familial - Familial is a genetic or inherited form which affects younger individuals.
  • Mariana Island - Mariana Island form is a rare form which is found in chamorro indian patients from Guam.

Statistics on ALS - Amyotrophic Lateral Sclerosis

There are about 20 to 30 thousand people suffering from ALS in USA alone. Every year, about 5 thousand are added. Speaking about the occurance of the disease, ALS is the most commonly occuring neuromuscular diseases the world over. People of all races and ethnic groups are affected by this fateful illness. This illness occurs in the people of age group 40 to 60 years. Sometimes there are incidences of even younger and much older people developing Amyotrophic Lateral Sclerosis disease. Men are affected more often than women.

ALS when affects someone, 90 to 95 percent of times, the reason is not found. In these people the disease occurs at random with no earlier associated risk factors and the affected person does not have any family history of the disease. The family members are also not considered to be at very high risk in developing ALS.

Rest 5 to 10 percent are inherited. This form of ALS is called as familial form and they tyically have a pattern of inheritance. According to a study, 20 percent of familial form affected cases have a genetic defect which leads to mutation of the enzyme SODI.

Causes of Amyotrophic Lateral Sclerosis

The causes of ALS unknown.

  • ALS may causes due to genetic factors. About 10% cases are caused due to genetic.
  • It causes when the death of motor neuron take place.

Symptoms of Amyotrophic Lateral Sclerosis

  • Muscle weakness and wasting
  • Trouble writing
  • Muscle twitches
  • Speech problems
  • Head drop due to weak spinal and neck muscles
  • Paralysis

Diagnosis of Amyotrophic Lateral Sclerosis

The neurological exam indicate the weakness on which area such as hip, shoulder etc. Some tests includes like that of

  • EMG - EMG test to see the proper functioning of the nerve.
  • CT & MRI scan - CT & MRI scan is done to find out the condition.
  • Respiratory test - To see the respiratory muscles are affected.
  • Blood test and Geneti Tests

Treatment of Amyotrophic Lateral Sclerosis

There is no known cure for ALS. To control symptoms some therapies are present like

  • Medications - Riluzole drug is the first medicine for ALS. Riluzole medicine help to prolong life. The medication help to prevent pain. Medications like baclofen and tizanidine may be used to relieve severe spasticity.
  • Physical and occupational therapy - A physical therapy includes exercises to maintain your muscle strength and range of motion as long as possible. An occupational therapy help you become accustomed to a brace, walker or wheelchair and make able to suggest devices such as ramps.
  • Speech therapy - A speech therapy help teach techniques to make your speech more clearly understood. In this speech therapy devices like speech synthesizers and computers are used to communicate.
  • Nutritional support - A dietitian suggest you to take foods that are nutritious and easy to swallow.
  • Respiratory therapy - Respiratory therapy help to prevent aspiration by regular suctioning and chest percussion.

Prevension of Amyotrophic Lateral Sclerosis

  • Patients require regular exercise and a physical therapy program.
  • Modify the patient's diet to prevent aspiration
  • Genetic counseling may be advised if there is a family history of ALS.

What is the prognosis of Amyotrophic Lateral Sclerosis?

In ALS progressive loss of ability to function and care for oneself. Death often occurs within 3 to 5 years of diagnosis. People with ALS are unable to stand or walk and get in or out of bed on their own, or use their hands and arms. Most people with ALS die from respiratory failure.

There is a requirement to research and study the motor neuron degeneration process which can further lead to a enhanced knowledge of ALS. It will also help in creating neuroprotective strategies for other similar illnesses. Researchers are hoping for the breakthrough in identifying the motor neuron degeneration process in ALS and then create therapies for restricting the disease or complete cure of the disease.