Amyotrophic Lateral Sclerosis

What is Amyotrophic lateral sclerosis ?

Amyotrophic lateral sclerosis is a condition that affects the nerve cells which are responsible for the voluntary muscle movements. This is a type of neurological disease where the patient’s condition aggravates gradually  until the nerve cells responsible for motor movements are paralyzed. Doctors and other health care associates fondly call it as Lou Gehrig ’s disease or ALS.

According to statistics, 1 out of 100,000 people will likely develop this disease. This usually happens when they reach 40 to 60 years old and men are more likely to develop the disease then women. The disease affects both the upper and lower neurons which are the brain, brainstem and the spinal cord. Then the neurons or nerve cells will die causing the patient to have body weakness. Once the disease progresses to the diaphragm area, the patient cannot breathe on their own without the use of a ventilator. With that, most of the patients  die because of respiratory failure within 3 to 5 years. There are however patients that survived up till 10 years.

Although amyotrophic lateral sclerosis attacks the voluntary muscles, the patient can still open their eyes, and their senses won’t be altered. They will still be able to have their sense of hearing, smell, taste and touch. But as the disease progresses, the patient will need all the help they need to go to the bathroom. The intelligence and cognitive function is also not affected but recent studies have shown that people with this disease can develop depression, memory loss and altered decision-making.

Amyotrophic Lateral Sclerosis Symptoms

On the onset of the problem, the patient will experience the following:

1. Dysphagia – this is a condition wherein the patient will have difficulty swallowing food.
2. Difficulty forming or words or dysarthria
3. There are muscle spasms felt and sometimes, the patient cannot control when flexing their arms or legs.
4. Babinski reflex will also show. This is common in infants but not in adult. When a health care provider or anybody will write an inverted “J” at the back of the foot, the patient’s toe extends in upward motion and the other toes spreads out.
5. Limbs onset- this happens when the patient usually trips off when of stumbles down when walking or running.
6. Arm onset- the patient will find it difficult to do tasks like buttoning their shirt, writing, opening the locked door and many other tasks that require the use of hands.
7. Patients will also experience pseduobulbar affect. This means that the patient will exhibit uncontrolled smiling or crying and even laughing.
8. Hoarseness or voice changes
9. Drooling
10. Weight loss can also happen.

As the disease progresses, the early symptoms will worsen. This will make the patient’s life difficult and assistance is needed in walking or standing. The use of arms and legs are affected making it difficult to eat, chew, and sometimes swallow. This is a great risk for choking making the person at risk for aspiration pneumonia.

Some patients may develop dementia especially those with family history of dementia. Most of the patients will experience problems with word-formation, decision-making and others. Nocturnal hypoventilation or having difficulty breathing at night will also be experienced by patients with amyotrophic lateral sclerosis.

Amyotrophic Lateral Sclerosis Pictures

Picture 1 :  Normal muscle and atrophied or damaged muscle due to amyotrophic lateral sclerosis

Picture 2 : ALS patient experiencing  stiffness and muscle weakness as the initial symptom.

Amyotrophic Lateral Sclerosis Causes

The real cause of amyotrophic lateral sclerosis is still unknown. Most experts associate this with family history or genetics. According to them, an inherited defective gene on chromosome 21 can likely cause about 20 percent of the problem.

Another probable cause is the environmental factor wherein the diseases affect most people at the Western Pacific. This is associated with a neurotoxin which is mixed in food sometime in the 1960’s. The seeds of cycad, a tropical plant are the said culprit.

Amyotrophic Lateral Sclerosis Diagnosis

Once the patient is admitted, a details patient history as well we family history is gathered. Then tests and exams will be done to conclude that the patient has amyotrophic lateral sclerosis. The diagnostic tests may be many because the disease can also be associated with other conditions.

1. Thorough neurologic exam is done with intervals to see asses the symptoms like muscle weakness, atrophy and the hyperreflexia. The doctor will also check if the patient has been clumsy or if emotional incontinence is present. This is observed by excessive laugh or cry.
2. EMG or electromyography. This is a test which records the electrical activity of the muscles.
3. NCV or nerve conduction velocity. This test also sees abnormalities when it comes to nerve functions.
4. Magnetic resonance imaging or MRI- this is a popular diagnostic test wherein detailed images of the radio waves of the brain’s activity. This gives the physician a clear picture of which part of the neurologic system is the problem. Whether it is on the brainstem, the brain itself or the spinal cord.
5. Blood and urine samples will also be taken so that it eliminates other problems that associates with the same symptoms.

Amyotrophic Lateral Sclerosis Treatment

Up to the present, there is still no known cure for the disease. But the Food and Drug Administration approved Riluzole or Rilutek. This drug is known to decrease the damage on the motor neurons. According to studies, this drug delays the need for the patient to be dependent on the ventilator. Physicians however always remind their patients that this does not cure nor reverse the damage done by the disease. And patients should be monitored frequently for liver problems and other side effects brought about by the drug.

Aside from that, supportive treatment is done to help the patient with the progressing disease. Medications are prescribed to reduce fatigue and ease on muscle cramps. Also cough medicines that reduces saliva and phlegm are also prescribed.

Presence of a medical professional must be seen as often to  assist the patient when moving, walking or standing up. Meals served should be appropriate for ease of swallowing. At some point; speech therapists are also needed in order to help the patient in speaking. Lastly a mechanical ventilator should also be on standby in health care facilities in cases of respiratory depression.

References:

http://www.ninds.nih.gov/disorders/amyotrophiclateralsclerosis/detail_ALS.htm

http://www.nlm.nih.gov/medlineplus/amyotrophiclateralsclerosis.html

http://www.alsa.org/about-als/diagnosing-als.html