Amyotrophic lateral sclerosis
Key points
- Amyotrophic lateral sclerosis, or ALS, is a disease of the motor nerve cells in the brain and spinal cord.
- Amyotrophic Lateral Sclerosis is characterized by progressive loss of motor nerves in the spinal cord and brain.
- Symptoms usually do not develop until after age 50.
- A neuromuscular examination indicates weakness, often beginning in one limb or area (such as shoulders or hips).
Alternative names
Lou Gehrig's disease; ALS
Definition
Amyotrophic lateral sclerosis, or ALS, is a disease of the motor nerve cells in the brain and spinal cord. It causes progressive loss of motor control.
Causes, incidence, and risk factors
Amyotrophic Lateral Sclerosis is characterized by progressive loss of motor nerves in the spinal cord and brain. In about 10% of cases, ALS is caused by a genetic defect. In other cases, the cause of the nerve deterioration is unknown.
ALS affects approximately 1 out of every 100,000 people.
Except for having a family member affected with the hereditary form of the disease, there are no known risk factors.
Symptoms
Symptoms usually do not develop until after age 50. Progressive loss of muscle strength and coordination eventually interfere with the ability to perform routine activities, such as going up steps, getting out of a chair, or swallowing. Occasionally, breathing or swallowing muscles may be the first to be affected.
As the disease progresses, more muscle groups are affected and patients become progressively incapacitated. There is no effect on the ability to think or reason.
Symptoms include:
- muscle weakness, decrease in muscle strength and coordination
o gradual onset
o progressively worsens
o commonly involves one limb initially (such as the hand)
o progresses to difficulty lifting, climbing stairs, and walking - paralysis
- muscle cramps
- voice changes, hoarseness
- speech impairment, slow or abnormal speech pattern
- difficulty swallowing, gags or chokes easily
- difficulty breathing (increasing effort required to breathe)
- head drop due to weak spinal and neck muscles
Additional symptoms that may be associated with this disease:
- muscle contractions
- muscle spasms
- muscle atrophy
- ankle, feet and leg swelling
- weight loss
- drooling
Signs and tests
Written and revised by
David Cohen, M.D.
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