Also known as ALS or Lou Gehrig's disease, amyotrophic lateral sclerosis is a rare neurological disease involving the breakdown, and eventual death, of neurons that control voluntary muscles. The brain loses the ability to initiate and control movement, often resulting in an inability to eat, speak, move, and even breathe. Approximately 5,000 people in the US are diagnosed with this condition per year, according to the ALS Foundation. There are treatments for ALS, but there is no cure.
Loss of coordination is one of the first warning signs of ALS. Reduced hand-eye coordination may start slowly, with the individual noticing relatively minor issues, such as the ability to grasp a hairbrush. Over time, the number of episodes increases in frequency, and in some cases coordination issues last for months before other symptoms begin to appear.
An early warning sign of ALS is cramps or muscle spasms, especially when they develop with other symptoms. Cramping, twitching, and atrophy of the muscles occur when spinal and brain stem motor neurons deteriorate. This particular symptom typically begins after the loss of hand-eye coordination and can last through many of the disease's stages.
ALS almost immediately begins to wreak havoc on the nervous system. Motor neurons that tell muscles to move begin to die, and as a result, individuals lose control of and strength in their muscles. The degeneration of neurons leads to loss of muscle mass, too. Due to the lack of muscle mass, people with ALS often require wheelchairs even before they lose the ability to walk.
Laryngeal dysfunction can occur when the loss of neurons affects the bulbar nerves. Individuals with ALS often experience changes in voice pitch, usually resulting in speaking at a lower register. This common symptom typically coincides with hand-eye coordination issues.
Slurring of speech happens for a different reason than the change in vocal pitch. This symptom occurs when the person is no longer capable of properly moving muscles involved with speech, including the lips and tongue. This is generally referred to as dysarthria, the inability to speak correctly due to loss of motor function. The individual affected may find it difficult to pronounce words properly.
Some people with ALS experience significant mood swings that can result in uncontrollable laughter or crying, known as the pseudobulbar affect. Although it is not completely understood why this occurs, it is believed that when upper motor neurons, located in the cerebral cortex and brain stem, deteriorate, the disrupted neural pathways result in a deficiency of inhibition control. Emotions can come on suddenly, without warning, and often without triggers, lasting only a few moments or up to an hour. Prescription medications can help control the onset and amplification of emotions.
Problematic breathing does not occur immediately in all people with ALS, but most experience it eventually. As muscles deteriorate, those responsible for bringing air in and out of the lungs are also affected. An individual may first develop shortness of breath and an inability to breathe in deeply. This can progress to collapsed lungs and a continuous need for a breathing machine.
Trouble walking is a common ALS symptom usually seen in later stages of the disease. Initially, an individual may experience weakness in the legs, which gradually worsens as the disease continues to cause muscle degradation. There are many possible causes of weakness in the legs; however, most people with ALS experience other symptoms before this one peaks.
Swallowing problems are another late symptom of ALS. The issue can make it difficult to eat and drink, which makes choking a constant danger as it becomes difficult even to swallow saliva. This symptom is caused by the lack of control of the muscles used when swallowing. Primary doctors may work with dietitians to devise meal plans that ensure individuals with ALS continue to get proper nutrition.
People with advanced ALS often experience an inability to control the muscles in the neck, resulting in a condition called dropped head syndrome. The muscles at the back of the neck weaken, which is why people with ALS often require neck braces.
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