What Is Amyotrophic Lateral Sclerosis ALS

what is amyotrophic lateral sclerosis als

Amyotrophic lateral sclerosis, or known as ALS, motor neuron disease, or upper and lower motor neuron disease, is a severe neurological disorder in brain and spinal cord that is characterized by disorders of voluntary muscle movement such as muscle weakness, permanent disability, until death. It is also called Lou Gehrig's disease because there was a basketball player named Lou Gehrig who died of this disease. Amyotrophic lateral sclerosis is a very uncommon disease who occurs at 5 of 100,000 people in the world. The risk exact causes of amyotrophic lateral sclerosis is still unknown, but generally it is a hereditary disease. Researchers also found that amyotrophic lateral sclerosis is caused by gene mutation, bad immune response, and chemical substance imbalance such as too much glutamate.

Symptoms of amyotrophic lateral sclerosis (ALS)
Symptoms usually start after age 50. As quoted from Mayo Clinic, early symptoms of ALS are:

1. Start with weakness in the upper extremities (clumsiness) 
2. Weakness in the lower extremities (leg, feet, ankles), footdrop
3. Slurred speech, inability in swallowing
4. Twitching in tongue, shoulders, and arms
5. Generally muscle cramps

Further symptoms of amyotrophic lateral sclerosis are:

1. Paralysis
2. Difficulty of breathing
3. Difficulty of swallowing
4. Progressive muscle weakness, difficulty of walking, lifting, etc
5. Head drop (neck muscle weakness)
6. Muscle cramps
7. Speech disorder

How to diagnose amyotrophic lateral sclerosis ALS?

1. Physical examination
2. Medical history
3. Blood test, to rule out another diseases that have similar symptoms
4. Electromyography (EMG): to record muscle's electrical activity.
5. Muscular biopsy by a pathologist.
6. Head CT-scan and MRI, lumbal puncture, to rule out another brain and nervous disorders.

Treatment

The goals of treatment in ALS patients are to slow the progression of the disease, and make the patients more comfortable and independent in doing basic daily activities.

1. Pharmacologic. 
• Riluzole (Rilutek) is recommended by FDA to slow the progression of ALS. It reduces the levels of glutamate in the brain
• Symptomatic therapies to cure: pain, muscle cramps, fatigue, depression, constipation, etc
2. Physical therapy: to maintain cardiovascular and neuromuscular fitness.
3. Speech therapy: to gain alternative methods of communication, so the ALS patients can still be capable in communicating.
4. Occupational therapy: find devices to support daily activities (if needed) such as wheelchair, walker, etc.