Lou Gehrig's disease, often called as Amyotrophic Lateral Sclerosis (ALS), is a progressive degenerative disorder that affects the brain and spinal cord. Consequently, the patient loses control over the voluntary muscles. The most commonly affected nerves include those controlling the limb movements, speech, and swallowing.
The disease is named after a famous baseball player, Lou Gehrig who was diagnosed with the condition in 1939. In 2014, several famous personalities including Justin Bieber, Usain Bolt, etc. have participated in the ice bucket challenge to promote awareness of amyotrophic lateral sclerosis.
The Lou Gehrig's disease symptoms which are produced in the initial stages include:
If the disease starts in the limbs, the patient will have difficulty with writing or buttoning a shirt, or will have a tripping or stumbling feel while walking or running.
As the disease progresses, the weakness spreads to the entire body. Eventually, the patients lose their ability to walk or even stand, use their hands and arms.
Complications which occur with the disease progression are as detailed below:
Eating problems: Malnutrition and dehydration can develop overtime due to difficulty with swallowing. Pneumonia can also occur due to the entry of food, and drinks into the lungs.As ALS is a devastating condition, it can be difficult for the person to cope up with the family and friends. But certain tips can be of help for such patients:
The exact cause is not known in most of the patients (sporadic), however, the ALS disease is believed to be caused due to mutations in the genes associated with the production of an enzyme called SOD1. As a result, the enzyme becomes toxic.
The other possible contributing causes of ALS include:
The following factors are identified to increase the risk of a person getting ALS:
Usually, the symptoms occur in people aged 50 to 60. However, it can also occur in early ages. Men have a slightly higher chance of getting ALS compared to women.
The diagnosis of ALS can take from weeks to months. Initially, your doctor may suspect that you have ALS if you show a progressive neuromuscular decline. Then the doctor will look for symptoms such as muscle weakness, wasting, twitching, and cramps. However, these symptoms may be present in other conditions. Therefore, a series of one or more of the following diagnostic tests are performed to rule out the other conditions and confirm ALS.
| Test type | Purpose of the test |
|---|---|
| Blood and urine tests | To evaluate your general health and nutrition |
| EMG (Electromyogram) | To evaluate the electrical activity of the muscles |
| MRI (Magnetic resonance imaging) | To identify which parts of the nervous system are affected |
| Nerve conduction studies | To test the nerve function |
| Muscle biopsy | To rule of muscle diseases |
| Genetic tests | May be performed if a family history of ALS is present |
Unfortunately, no treatment so far is available to cure Lou Gehrig disease. Treatments focus on slowing down the disease progression and help you to lead an independent life.
Medications: The only drug approved for treating ALS is riluzole. It reduces the production of glutamate.The other drugs prescribed for symptomatic treatment include those for:
The other therapies employed in the treatment of ALS are:
Nutritional support: Your family members will be instructed on how to meet your nutritional requirements and help you with swallowing difficulty.