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What is Amyotrophic Lateral Sclerosis?
Amyotrophic lateral sclerosis (ALS), some call this disease as Lou Gehrig's disease the name of the baseball player who died with this disease, is a rapidly progressive, invariably fatal neurological disease that attacks the nerve cells (neurons) responsible for controlling voluntary muscles.  In ALS, both the upper motor neurons in th brain and the lower motor neurons  in the spinal cord degenerate or die, ceasing to send messages to muscles. Unable to function, the muscles gradually weaken, waste away, and twitch. Eventually the ability of the brain to start and control voluntary movement is lost.  Individuals with ALS lose their strength and the ability to move their arms, legs, and body. When muscles in the diaphragm and chest wall fail, individuals lose the ability to breathe without ventilatory support.  The disease does not affect a person's ability to see, smell, taste, hear, or recognize touch, and it does not usually impair a person’s thinking or other cognitive abilities.  However, several recent studies suggest that a small percentage of patients may experience problems with memory or decision-making, and there is growing evidence that some may even develop a form of dementia.  The cause of ALS is not known, and scientists do not yet know why ALS strikes some people and not others.

CIDPUSA studied this disease and has found a connection between baseball players , football players getting this disease. Some scientist have said that there may be a connection between the grass they play on and fall on that ground may be contaminated with pesticide. These pesticide may have a role in causing this disease. This needs to be further investigated. Discussed in our E-book.

Is there any treatment?

Some new studies have come out which show that CIDP and ALS may be linked, there have been case reports that antiinflammatory and immununomodulating drugs have helped these cases. A possiable for cure for ALS has not been found but AL in case reports has improved. Please see the links of ALS from our home page. However, the FDA has approved the first drug treatment for the disease—riluzole.  Riluzole is believed to reduce damage to motor neurons and prolongs survival by several months, mainly in those with difficulty swallowing.  Other treatments are designed to relieve symptoms and improve the quality of life for people with ALS.  Drugs also are available to help individuals with pain, depression, sleep disturbances, and constipation.  Individuals with ALS may eventually consider forms of mechanical ventilation (respirators).  Some reports have recommended the use of antibiotics to help victims of ALS.

What is the prognosis?

Regardless of the part of the body first affected by the disease, muscle weakness and atrophy spread to other parts of the body as the disease progresses. Individuals have increasing problems with moving, swallowing, and speaking or forming words. Eventually people with ALS will not be able to stand or walk, get in or out of bed on their own, or use their hands and arms. In later stages of the disease, individuals have difficulty breathing as the muscles of the respiratory system weaken. Although ventilation support can ease problems with breathing and prolong survival, it does not affect the progression of ALS. Most people with ALS die from respiratory failure, usually within 3 to 5 years from the onset of symptoms. However, about 10 percent of those individuals with ALS survive for 10 or more years.

 

 
Muscle Nerve. 2005 Nov 30;33(3):356-362 [Epub ahead of print]  

A study of three patients with amyotrophic lateral sclerosis and a polyneuropathy resembling CIDP.

Echaniz-Laguna A, Degos B, Mohr M, Kessler R, Urban-Kraemer E, Tranchant C.

Departement de Neurologie, Hopital Civil de Strasbourg, 1 Place de l'Hopital, BP426, 67091 Strasbourg, France.

We report three patients with a syndrome that fulfilled clinical and laboratory criteria for definite chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) who failed immunosuppressive treatment and eventually developed progressive amyotrophic lateral sclerosis (ALS). Mean disease duration was 23 months (13-38) before death. Two patients had a family history of ALS without mutations of the SOD1 gene. Postmortem examination in one patient showed an endoneurial infiltration of mononuclear cells in lumbar roots and distal and proximal peripheral nerves, mainly around myelinated fibers, with demyelination and axonal loss, consistent with CIDP. The spinal cord revealed severe neuronal loss in the anterior horn, axonal loss in the corticospinal tract, and large numbers of phagocytes in the anterior and lateral tracts, indicative of ALS. Whether demyelinating polyneuropathy was coincident with ALS or was a cause or consequence of motor neuron degeneration in these patients remains to be elucidated. This unusual combination may provide an important clue in elucidating the pathogenesis of ALS in some patients. Muscle Nerve, 2006.

PMID: 16320313 [PubMed - as supplied by publisher]

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