Amyotrophic Lateral Sclerosis (ALS)
- Article
- 2021-03-02
Amyotrophic lateral sclerosis (ALS) is a rapidly progressing, deadly disease that affects the nerve cells (neurons) in the brain and spinal cord that control voluntary muscle movement. Our voluntary muscles produce movements such as walking, breathing, chewing and talking. Nerve cells called motor neurons - which connect the brain and spinal cord to the rest of the body - begin to degenerate and die, and stop sending messages to the muscles. The muscles gradually weaken, languish and vibrate, and the brain cannot initiate and control voluntary movements. Symptoms are usually first noticed in the arms and hands, legs or swallowing muscles. People with ALS lose their strength and can no longer move their arms and legs and keep the body upright. Some people are ultimately unable to breathe on their own. Although ALS doesn't 'Because usually a person's mind or personality is affected, several recent studies suggest that some people with ALS may develop cognitive problems related to word fluency, decision-making, and memory. Most cases of ALS happen without a known cause, while a small percentage of cases are inherited.
Therapy
No cure has yet been found for ALS. However, the drugs riluzole and edaravone have been approved by the Food and Drug Administration (FDA) to treat ALS. Riluzole extends lifespan by 2-3 months, but does not relieve symptoms. Edaravone may slow the clinical deterioration of the daily functioning of people with ALS. The FDA has also approved the NeuRx Diaphragm Pacing System, which uses implanted electrodes and a battery pack to contract the diaphragm (respiratory muscle) to help certain individuals with ALS before severe respiratory failure occurs. Other treatments are designed to relieve symptoms and improve the quality of life for people with ALS. Medicines are available to help people with spasticity, pain, panic attacks, and depression. Physical therapy, occupational therapy, and rehabilitation can help prevent joint immobility and slow muscle weakness and atrophy. Individuals with ALS may eventually consider forms of mechanical ventilation (breathing equipment).
Prognosis
Regardless of which part of the body was first affected by the disease, muscle weakness and atrophy spread to other parts of the body as the disease progresses. Individuals increasingly have problems moving, swallowing and speaking or forming words. Eventually, people with ALS will no longer be able to stand or walk, get in or out of bed alone, or use their hands and arms. In later stages of the disease, people have difficulty breathing because the muscles of the respiratory system weaken. Although ventilator support can relieve respiratory problems and prolong survival, it does not affect the progression of ALS. Most people with ALS die of respiratory failure, usually within 3 to 5 years of the onset of symptoms. However, about 10 percent of those with ALS survive 10 or more years.
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