CLINACASE

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ID: 215
Category: Neurological Disorder
CreatedBy: 1
UpdatedBy: 1
createdon: 14 Jul 2017
updatedon: 16 May 2023

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Author: Khoa Tran
Published Jul 14, 2017
Updated May 16, 2023

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Amyotrophic Lateral Sclerosis (ALS)

Etymology and Pronunciation

Amyotrophic (a-my-o-troh-fik)
a- - Greek prefix for "without"
myo- - Greek for "muscle"
trophic - Greek for "nourishment"

Lateral (lat-er-uhl)
lateralis - Latin for "belonging to the side"
latus - Latin for "side"
-alis - Latin suffix for "related to" or "pertaining to"

Sclerosis (skluh-roh-sis)
sklerosis - Greek for "hardness" or "induration"

History of Amyotrophic Lateral Sclerosis (ALS)

Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig's disease. The name "Lou Gehrig's disease" comes from the famous American baseball player Lou Gehrig, who was diagnosed with ALS in 1939. Gehrig's diagnosis and subsequent decline in health were widely publicized in the media, bringing greater attention to the disease and helping to raise awareness about its devastating effects. After Gehrig's death in 1941, the disease came to be commonly known as "Lou Gehrig's disease" in the United States, particularly among baseball fans and the general public.

While the name "Lou Gehrig's disease" is still used today, particularly in the United States, it is important to note that the condition is officially known as amyotrophic lateral sclerosis worldwide.

Amyotrophic lateral sclerosis was first described in 1869 by the French neurologist Jean-Martin Charcot. Charcot observed patients with a combination of muscle weakness, stiffness, and wasting, along with other neurological symptoms. He initially called the condition "amyotrophic lateral sclerosis" because it affected the lateral columns of the spinal cord, which are responsible for the motor control of the muscles.

In the years following Charcot's initial description, other physicians and researchers began to investigate the condition further. One of the key figures in this research was a British physician named James Parkinson, who is best known for his description of Parkinson's disease. Parkinson's observations of patients with ALS helped to refine the clinical features of the condition, including the progressive weakness and wasting of muscles, and the eventual paralysis of affected individuals.

Over time, researchers began to identify different forms of ALS, including familial forms that appeared to be inherited, and sporadic forms that occurred without any known genetic or environmental factors. Advances in genetics and neuroscience in the 20th century led to further understanding of the pathophysiology of the disease, including the role of mutations in the SOD1 gene and other genes that contribute to the development of the disease.

Today, ALS remains a complex and challenging condition to understand and treat. While there is still no cure for ALS, there are a variety of treatments and therapies that can help to manage symptoms and improve quality of life for affected individuals.

Modern Understanding of Amyotrophic Lateral Sclerosis (ALS)

Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, is a progressive and debilitating neurological disorder that affects the nerve cells responsible for controlling muscle movement. ALS gradually damages and destroys these nerve cells, leading to a gradual weakening and wasting away of muscles throughout the body.

The symptoms of ALS can vary widely from person to person, but typically begin with muscle weakness and stiffness, usually in the hands, arms, and legs. As the disease progresses, these symptoms may spread to other parts of the body, including the torso, face, and throat. Other common symptoms include difficulty with speech and swallowing, muscle twitching and cramping, and eventually, complete paralysis.

Causes of Amyotrophic Lateral Sclerosis (ALS)

The exact cause of ALS is unknown, but it is believed to be a combination of genetic and environmental factors. In some cases, it can be caused by inherited genetic mutations, while in others, it appears to develop spontaneously with no known cause.

Treatments for Amyotrophic Lateral Sclerosis (ALS)

There is currently no cure for ALS, and treatments are focused on managing symptoms and improving quality of life. This may include medication to relieve muscle cramps and stiffness, physical therapy to improve muscle strength and flexibility, and respiratory therapy to help with breathing difficulties.

While ALS is a devastating disease, there is ongoing research to better understand its causes and develop more effective treatments. In the meantime, support from family, friends, and healthcare professionals can be crucial for those living with ALS and their loved ones.

Symptoms

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Difficulty walking or doing normal daily activities
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Tripping and falling
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Weakness in your legs, feet or ankles
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Hand weakness or clumsiness
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Slurred speech or trouble swallowing
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Muscle cramps and twitching in your arms, shoulders and tongue
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Inappropriate crying, laughing or yawning
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Cognitive and behavioral changes
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Muscle Weakness

Confirmation Tests

- Nerve conduction test
- Nerve biopsy
- MRI
- Spinal fluid analysis
- Muscle biopsy

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