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Amyotrophic Lateral Sclerosis

Definition


Disease: Amyotrophic Lateral Sclerosis Amyotrophic Lateral Sclerosis
Category: Neurological diseases
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Disease Definition:

Amyotrophic lateral sclerosis, also called Lou Gehrig's disease, is a serious neurological disease that causes muscle weakness, disability and death.

 

Stephen Hawking, the famous English theoretical physicist and cosmologist, is severely disabled by this condition.  Hawking is markedly different from typical ALS in that his form of ALS would make for the protracted case ever documented.

ALS occurs in 1 to 3 people per 100,000 people worldwide. Its inherited form occurs in almost 5-10% of the cases. In the majority of cases, the reason of its occurrence is unknown.

Usually, ALS begins with slurring of speech or muscle twitching and weakness in an arm or leg and it ultimately affects the ability to control the muscles needed to move, eat, speak and breathe.

Work Group:


Prepared by: Scientific Section

Symptoms, Causes

Symptoms:

Some of the early signs and symptoms of ALS may be:

 

  • Trouble swallowing or slurring of speech.
  • Difficulty lifting the front part of the foot and toes (footdrop).
  • Clumsiness or hand weakness.
  • Weakness in the leg, feet or ankles.
  • Muscle cramps and twitching in the arms, shoulders and tongue.


As the disease advanced, the muscles will become progressively weaker until they're paralyzed. It begins in the hands, feet or limbs and then spreads throughout the body and eventually affects chewing, swallowing, speaking and breathing.

Causes:

In ALS, the muscles progressively weaken and begin to waste away because the nerve cells that control the movement of muscles gradually die. Up to 1 in 10 cases of ALS is inherited. But the remainder appears to occur randomly.

Autoimmune responses:
In some cases, a person's immune system targets and attacks some of its own normal cells. It has been speculated that such antibodies could trigger the process that results in ALS.

Free radicals:
In the inherited form of ALS, a mutation occurs in the gene that is responsible for producing a strong antioxidant enzyme that protects the cells from damage caused by free radicals, which are the byproducts of oxygen metabolism.

Glutamate:
In the spinal fluid of people with ALS, there are higher than normal levels of glutamate, a chemical messenger in the brain. For some nerves, this could be toxic.

Complications

Complications:

As the disease progresses, some of the  complications that may occur include:

Eating problems:
People with ALS are at a high risk of aspirating food, liquid or secretions into their lungs, causing pneumonia, but a feeding tube can reduce these risks. Additionally, because the muscles that control swallowing are affected by ALS, people with this condition are known to develop dehydration and malnutrition.

Dementia:
People with ALS are at a higher risk of developing:

 

 

  • Alzheimer's disease
  • Frontotemporal dementia


Breathing problems:
There are some devices that assist in breathing, which are similar to devices used by people who have sleep apnea. Some people, in the latter stages of ALS choose to have a tracheostomy and use the full-time help of a respirator that inflates and deflates their lungs. ALS eventually paralyzes the muscles needed to breathe, and the most common cause of death for people with ALS is respiratory failure, which usually happens within three to five years after symptoms begin.

Treatments:

Treatments for amyotrophic lateral sclerosis involve efforts to slow the progression of symptoms in order to make the patient more comfortable and independent because there’s no reversing the course of this disease.

Some of the treatment options may be:

MEDICATIONS:
Riluzole  is the only medicine that is approved by the Food and Drug Administration for slowing ALS, which in some people appears to slow the disease’s progression, perhaps by reducing levels of glutamate, which is a chemical messenger in the brain.

Someone with ALS may also be prescribed medications to treat:

 

  • Constipation
  • Pain
  • Excessive salivation
  • Muscle cramps
  • Fatigue
  • Depression
  • Excessive phlegm


THERAPY:

Occupational Therapy:
In this therapy, the patient will be helped to become accustomed to a wheelchair, walker or brace. They may also be suggested devices that make it easier for them to get around, such as ramps.

Physical Therapy:
To maintain their muscle strength and range of motion as long as possible and preserve a sense of independence, a person with ALS will be recommended low-impact exercises.

Speech Therapy:
As the disease progresses, communication will become an issue because ALS affects the muscles that are used to speak. A speech therapist can teach techniques to make the patient’s speech more clearly understood. He/she can also recommend devices that may help the patient communicate, such as speech synthesizers and computers.

Prognosis:

Not Available

Expert's opinion

Expert's Name:
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Expert's opinion:

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Clinical Trials:

Not Available

 

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