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What is ALS?
ALS is a rare, progressive disorder characterized by the loss of motor neurons. Motor neurons are nerve cells that enable muscle movement. These neurons are located in the brain and spinal cord. The motor neurons that reside in the brain are called UPPER motor neurons, while those found in the spinal cord are the LOWER motor neurons. The loss of upper motor neurons results in muscle stiffness, as well as slow movements and loss of fine motor dexterity. When lower motor neurons are injured, weakness, atrophy (loss of muscle mass), fasciculations (muscle twitching) and cramps are the typical symptoms.
In most cases, the disease begins in the limbs. In about one third of people, the symptoms start in what’s called the “bulbar” region, primarily affecting speech and swallowing. Rarely, the disease can start by affecting the breathing muscles. Up to half of the people with the disease may experience, at some point, difficulty with particular cognitive functions such as language (e.g. word finding) and executive function (e.g. complex decision making). The cognitive decline may be severe in a minority, meeting the criteria for dementia.
What causes ALS?
In 10-15% of cases, ALS is caused by a gene mutation. Most often, those patients with a genetic cause of their disease also report a family history of ALS and/or frontotemporal dementia (FTD). But around 5% of patients without a family history may also have disease caused by a genetic mutation. The cause of non-genetic (or “sporadic”) ALS is not well established. Advancing age is a risk factor, with the probability of disease increasing after age 40, peaking at age 72; the average age at onset is about 62. Smoking is probably also a risk factor.
Although many other possible other risk factors have been proposed (such as military service, head trauma and exposure to environmental toxins), none have been unequivocally proven to cause ALS. Whatever the cause of disease, the result appears to be the accumulation of abnormal proteins in motor neurons, resulting in cellular injury and ultimately cell death.
ALS Treatments & Symptom Management
There is currently no known cure for ALS. The two FDA-approved medications, riluzole (Rilutek) and edaravone (Radicava), offer modest benefits.
Despite the lack of a truly effective therapy, there are many management options, including for the myriad of symptoms that may result from the loss of motor neurons. It is therefore very important that ALS patients are treated by a team of experts, who are able to address the different ways in which the disease impacts a person living with ALS. Indeed, a multi-disciplinary approach to the care of ALS patients is well known to significantly improve the quality of life and survival. Click here to learn more about multi-disciplinary care in ALS, and click here to learn more about the Kessenich Family ALS Clinic at the University of Miami.
In the absence of a cure for ALS, we firmly believe that patients should have the opportunity to participate in clinical trials of experimental therapies, and to contribute to research into the underlying causes and biology of disease. Such research can only be done through a partnership between patients and clinicians/scientists. Click here to learn more about ALS research at the University of Miami ALS Center.
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