Amyotrophic Lateral Sclerosis

Amyotrophic Lateral Sclerosis

Abstract
Amyotrophic lateral sclerosis (ALS), also referred to as Lou Gerhig’s disease and Charcot disease, is a disorder that involves the death of neurons. ALS is characterized by stiff muscles, the twitching of muscles and gradually worsening weakness due to muscle deterioration. This results difficulty of speaking, swallowing and eventually breathing. Many who contract this disease die to respiratory failure. When was this disease discovered? Is there a cure? We will soon find out. ALS usually starts around age 60, inherited cases usually start around age 50. There is no known cause of ALS. About 5-10% of cases are inherited from parents. About half of these genetic cases are due to one of two specific genes. It results in the death of the neurons that control voluntary muscles. The diagnosis is based on a person's signs and symptoms with testing done to rule out other potential causes.
Descriptions of the disease date back to 1824, by a man named Charles Bell. In 1869 connections between the symptoms and the neurological problems were first described by Jean
Charcot, who in 1874 began using the term amyotrophic lateral sclerosis. ALS became well known in the United States when it affected famous baseball player Lou Gehrig, and 20th century Stephen Hawking. The start of ALS may be so subtle that the symptoms are overlooked. The earliest signs of ALS are obvious weakening of muscles or muscle atrophy. Other symptoms may include slurred speech, difficulty swallowing, cramping, or stiffness of affected muscle.
The parts of the body affected by early symptoms of ALS depend on which motor neurons are damaged. Around 75% of people that contract ALS first experience weakness of an arm or leg.
Awkwardness while running or walking or tripping and stumbling maybe be experienced along with a “dropped foot” which gently drags on the ground when you walk. Difficulty while buttoning a shirt or putting a key in a lock will occur, sometimes the symptoms are confined to one limb for the duration of the illness. Individuals with ALS may ultimately lose all voluntary muscle movement although bladder and bowel function and the muscles responsible for eye movement are usually spared. Cognitive function for the most part remains intact, although about 5% of people develop dementia . The sensory nerves are generally unaffected meaning that most people do not lose the ability of sight, hearing, taste, touch, and smell. Over time people experience increasing difficulty moving, swallowing, and speaking or forming words.

Exaggerated muscle reflexes also began to happen including overactive gag reflex. Around 15-
45% of people also experience a neurological disorder known as emotional lability, which consists of uncontrollable laughter, crying, and smiling. The median survival rate from onset to death is around 39 months, only 4% survive longer than 10 years. Stephen Hawking has lived 50 years with ALS, guitarist Jason Becker has been living with ALS since 1989, but these are both considered rare cases. Difficulty with chewing and swallowing increases the chance of choking and aspiration pneumonia. As the diaphragm and muscles of the rib cage that support breathing weaken, measures of lung function such as inspiratory pressure diminish. This may occur before significant limb weakness is apparent. When the muscles that assist in breathing weaken, use of ventilatory assistance such as biphasic cuirass ventilation (BVC), may be used to aid breathing Such devices artificially inflate the person's lungs from various external sources that are applied directly to the face or body. When muscles are no longer able to maintain oxygen and carbon dioxide levels, these devices may be used full-time. BCV

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