Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease)

Amyotrophic lateral sclerosis (ALS) is a degenerative disease that affects the nerve cells in the brain and the spinal cord that control muscle function, causing weakness and muscle atrophy. ALS also is known as Lou Gehrig's disease, in memory of the baseball hall of famer whose career ended in 1939 due to the condition.

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Symptoms

Early symptoms of amyotrophic lateral sclerosis may be very subtle.

Early signs include:

  • Generalized muscle weakness in the hands, arms, legs or feet
  • Tripping or dropping items
  • Difficulty lifting items
  • Trouble using hands for everyday activities

As the disease progresses, patients may experience:

  • Increased muscle weakness and twitching
  • Cramps and stiffness in the hands and feet
  • Slurred speech
  • Shortness of breath
  • Difficulty chewing, swallowing or breathing
  • Complete paralysis


Causes and Risk Factors

In a majority of cases, the cause of ALS is unknown. About 5 to 10 percent of cases are attributable to familial ALS (FALS), which is seen in families where two or more members have the condition.

ALS generally affects adults, with most patients diagnosed between the ages of 40 and 70. The disease is more common in men than women, although the distribution becomes more equal in older patients.  

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Diagnosis

There is no singular definitive test to diagnose ALS. Diagnosis is based on an expert evaluation of a combination of pure motor symptoms and signs that involve both the central nervous system and peripheral motor nerves. Because ALS symptoms can be similar to those of other diseases and disorders, physicians will order tests to exclude other conditions before confirming a diagnosis of ALS.

An electromyography (EMG), which measures electrical activity in muscles, is used to determine whether the problem is nerve-related or muscle-related. Measurement of nerve conduction velocity (NCV) may show abnormalities that can indicate another disease. While magnetic resonance imaging (MRI) results are often normal in ALS patients, they may indicate an alternative diagnosis.

Based on the results of diagnostic tests, a physician may order other tests including blood and urine studies, thyroid and parathyroid hormone level tests, lumbar puncture, X-rays and muscle and/or nerve biopsies.

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Treatments

Although there is no cure for ALS, there are treatments available that may help slow the progression of the condition. Since ALS affects many major functions of the body, a multidisciplinary team approach, such as the one employed by the ALS Program in the Cedars-Sinai Department of Neurology, can offer the best overall treatment.

Drug therapy, physical therapy, occupational therapy, respiratory therapy and speech therapy may help ALS patients.

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