Amyotrophic Lateral Sclerosis 
Also known as:  ALS or Lou Gehrig's Disease
What is ALS?
 What do the letters ALS stand for?

 ALS stands for Amyotrophic Lateral Sclerosis. Like many medical terms it comes from Greek words.
                   A—without
                   Myo—muscle
                   Trophic—nourishment
                   Lateral—side (of the spinal cord)
                   Sclerosis—hardening or scarring

ALS, a motor neuron disease, was first identified in 1869 by the noted French neurologist Jean-Martin Charcot. Although the cause of ALS is not completely understood, the 1990's have brought a wealth of new scientific understanding about the physiology of this disease. 

 Lou Gehrig, with whom ALS is most commonly associated, first brought national and international attention to the disease back in 1939 when he abruptly retired from baseball after being diagnosed with ALS. 

However, ALS is not just Lou Gehrig's disease and it knows no boundaries. The disease has cut short the lives of such notable and courageous individuals as Hall of Fame Pitcher, Jim "Catfish" Hunter; actor, Michael Zaslow; creator of Sesame Street, Jon Stone; actor, David Niven; boxing champion, Ezzard Charles; Pro Football Player, Glenn Montgomery and Senator, Jacob Javits. 

As motor neurons degenerate, they can no longer send impulses to the muscle fibers that normally result in muscle movement. Early symptoms of ALS often include increasing muscle weakness, especially involving the arms and legs, speech, swallowing and breathing. When muscles no longer receive the messages from the motor neurons that they require to function, the muscles begin to atrophy (waste away). Limbs begin to look "thinner" as muscle tissue atrophies. 
Although the cause of ALS is not completely understood, the 1990's have brought a wealth of new scientific understanding regarding the physiology of this disease.
Initial Symptoms of the Disease
At the onset of ALS the symptoms may be so slight that they are frequently overlooked. With regard to the appearance of symptoms and the progression of the illness, the course of the disease may include the following: 
  • muscle weakness in one or more of the following: hands, arms, legs or the muscles of speech, swallowing or breathing
  • twitching (fasciculation) and cramping of muscles, especially those in the hands and feet
  • impairment of the use of the arms and legs"thick speech" and difficulty in projecting the voice
  • in more advanced stages, shortness of breath, difficulty in breathing andswallowing 
The initial symptoms of ALS can be quite varied in different people. One person may experience tripping over carpet edges, another person may have trouble lifting and a third person's early symptom may be slurred speech. The rate at which ALS progresses can be quite variable from one person to another. Although the mean survival time with ALS is three to five years, many people live five, ten or more years. In a small number of people, ALS is known to remit or halt its progression, though there is no scientific understanding as to how and why this happens. Symptoms can begin in the muscles of speech, swallowing or in the hands, arms, legs or feet. Not all people with ALS experience the same symptoms or the same sequences or patterns of progression. But, progressive muscle weakness and paralysis are universally experienced. 

Muscle weakness is a hallmark initial sign in ALS, occurring in approximately 60% of patients. Early symptoms vary with each individual, but usually include tripping, dropping things, abnormal fatigue of the arms and/or legs, slurred speech, muscle cramps and twitches and/or uncontrollable periods of laughing or crying. 

The hands and feet may be affected first, causing difficulty in lifting, walking or using the hands for the activities of daily living such as dressing, washing and buttoning clothes. 

As the weakening and paralysis continue to spread to the muscles of the trunk of the body the disease, eventually affects speech, swallowing, chewing and breathing. When the breathing muscles become affected, ultimately, the patient will need permanent ventilatory support in order to survive. 

Since ALS attacks only motor neurons, the sense of sight, touch, hearing, taste and smell are not affected. For many people, muscles of the eyes and bladder are generally not affected. 

For the vast majority of people, their mind and thoughts are not impaired and remain sharp despite the progressive degenerating condition of the body. 

Three classifications of ALS
  • Sporadic - the most common form of ALS in the United States - 90 to 95% of all cases. 
  • Familial - occurring more than once in a family lineage (genetic dominant inheritance) accounts for a very small number of cases in the United States - 5 to 10% of all cases. 
  • Guamanian - an extremely high incidence of ALS was observed in Guam and the Trust Territories of the Pacific in the 1950's. 


The most common form of ALS in the United States is "sporadic" ALS. It may affect anyone, anywhere. 

"Familial" ALS (FALS) means the disease is inherited. Only about 5 to 10% of all ALS patients appear to have genetic or inherited form of ALS. In those families,  there is a 50% chance each offspring will inherit the gene mutation and may develop the disease. 

Diagnosis of ALS
ALS is a very difficult disease to diagnose. To date, there is no one test or procedure to ultimately establish the diagnosis of ALS. It is through a clinical examination and series of diagnostic tests, often ruling out other diseases that mimic ALS, that a diagnosis can be established. A comprehensive diagnostic workup includes most, if not all, of the following procedures: 
  • electrodiagnostic tests including electomyography (EMG) and nerve conduction velocity (NCV)  blood and urine studies including high resolution serum proteinelectrophoresis, thyroid and parathyroid hormone levels and 24 hour urinecollection for heavy metals
  • spinal tap
  • x-rays, including magnetic resonance imaging (MRI) 
  • myelogram of cervical spine
  • muscle and/or nerve biopsy thorough neurological examination
These tests are done at the discretion of the physician, usually based on the results of other diagnostic tests. 

There are many diseases that have some of the same symptoms as ALS and many of these conditions are treatable. 

Information obtained for this web page has been obtained from the
The ALS Association (ALSA) with permission of the webmaster.
How You Can Help?
The "How You Can Help" section of The ALS Association (ALSA) outlines the many ways you can help send a message of hope to the tens of thousands of men and woman who have ALS or will be diagnosed with this cruel disease. All those with ALS and their loved ones will thank you.
Links
Paul's PALS- ALS Support Reaching Out To You
A very nice website put together by Paul Johnson, who is an R.N. and a caregiver to his mother who has ALS. 

The latest medical news and information for patients or friends/parents of patients diagnosed with ALS (Lou Gehrig's) Disease 
MDA Diseases
Your source for information and news about ALS.  Updated regularly. 

Amyotrophic Lateral Sclerosis (ALS) 
or Lou Gehrig's disease
Drug InfoNet
Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease) FDA Approval of the First Treatment for ALS - Orbis Radio News Special Report. 
ALS / Lou Gehrig's Caregivers' Community
Web of Care provides information, support and products for people caring for a loved one with ALS at home. 
A Slide Presentation
ALS (Lou Gehrig.s Disease) 
Slide 1 of 15. 

 

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