Hello, it’s your health buddy! Let’s feel better together. Today, I’m here to share some symptom insights and helpful tips about Amyotrophic Lateral Sclerosis (ASL) or Lou Gehrig's Disease. Let’s dive in!
A condition that selectively destroys only motor neurons
Definition
ALS, also known as Lou Gehrig’s disease, is a condition in which only motor neurons are selectively destroyed. It progressively damages both the upper motor neurons (in the cerebral cortex) and the lower motor neurons (in the brainstem and spinal cord).
The clinical symptoms typically begin with slowly progressing weakness and muscle wasting in the limbs. As the disease advances, it eventually leads to respiratory muscle paralysis and death within a few years.
ALS occurs in about 1 to 2 people per 100,000 annually. The onset of the disease increases in people in their late 50s, and it is 1.4 to 2.5 times more common in men than in women.
Causes
The exact cause of ALS is still unknown, but several theories have been proposed. Around 5–10% of all ALS cases are familial (inherited), and about 20% of those familial cases show a genetic mutation on chromosome 21.
So far, mutations in eight different genes have been identified as causes of familial ALS.
For sporadic ALS (non-inherited), a process called excitotoxicity—cell death triggered by excessive stimulation—appears to play a key role.
Other possible causes include viral infections and exposure to environmental toxins, though no direct evidence has been confirmed yet.
Symptoms
ALS is a fatal disease that starts with gradually worsening weakness and muscle wasting in the limbs, eventually progressing to respiratory muscle paralysis and death within a few years.
As upper motor neurons are destroyed, damage spreads through motor pathways like the corticospinal and corticobulbar tracts. This causes symptoms in areas such as the face, trunk, and limbs depending on which part of the brainstem and spinal cord is affected. These symptoms are collectively known as upper motor neuron syndrome.
At the same time, damage to the lower motor neurons located in the anterior horns of the spinal cord causes additional symptoms known as lower motor neuron syndrome.
A key symptom includes partial contraction of the tongue muscles, which can cause choking, coughing while eating, and a higher risk of aspiration pneumonia (when food or liquids accidentally enter the lungs).
Breathing difficulties may occur due to weakening of the diaphragm and intercostal muscles. When the diaphragm is weakened, especially while lying down, the abdominal organs can press up into the chest cavity and make breathing harder.
Some patients may also experience cognitive impairment.
Diagnosis and Testing
ALS is primarily diagnosed based on clinical symptoms. A neurologist will look for signs of both upper and lower motor neuron involvement to make a clinical diagnosis.
Tests like nerve conduction studies and electromyography (EMG) are used to confirm ALS.
To rule out other neurological or muscular disorders with similar symptoms, imaging such as MRI scans of the brain or cervical spine may be done, as well as muscle biopsies.
Various blood tests may also be performed to help identify potential causes.
Treatment
Although multiple drugs are being developed based on the known mechanisms and progression of ALS, no treatment has yet shown definitive effectiveness.
The only FDA-approved medication currently is riluzole (brand name Rilutek), which can prolong survival by a few months. However, it has not been proven to improve quality of life or restore muscle strength.
Prognosis and Complications
The average life expectancy after diagnosis is 3 to 4 years. However, about 10% of patients show a milder progression and may survive for more than 10 years.
Research is ongoing to identify the factors that contribute to long-term survival.
Your health buddy is here for you. Wishing you a healthy and happy day ahead. Stay well!💖
This content is for informational purposes only and does not hold any individual or entity legally responsible. For accurate diagnosis and treatment of symptoms, please visit a nearby medical institution. This does not represent my final medical opinion and may vary depending on specific circumstances and evolving medical perspectives.
