Amyotrophic Lateral Sclerosis
Amyotrophic Lateral Sclerosis (ALS) is a disease that affects the nerve cells in the brain and spinal cord, specifically the motor neurons. Motor neurons carry signals from the brain and spinal cord to all of the muscles in your body. When a person has ALS, they are not able to generate enough motor neurons, and the brain cannot then initiate and control muscle movement. After some time, when the disease gets progressively worse, the patient has difficulty speaking, swallowing, breathing, etc. These kinds of tasks are essential to the body, so when it gets to a certain point, the patient dies. There are several other names for this disease, such as Lou Gehrig’s disease, and Motor Neurone Disease (MND).
Signs and Symptoms
The earliest symptoms of ALS are weakness of the muscles, which is to be expected. These types of symptoms can involve having trouble swallowing, cramping, or stiffness of certain muscles. As the disease progresses, patients lose the ability to control all voluntary movements. Generally, sensory nerves and the autonomic nervous system are unaffected, meaning that individuals with ALS can hear, see, touch, smell, and taste properly. The types of muscles you are able to control by the early symptoms, all depends on which motor neurons are damaged first. The most common motor neurons that get damaged early on are the ones called “limb onset,” which have to do with the arms and legs. As expected, people with “limb onset” experience trouble when walking and running, such as stumbling, tripping, or dragging of the foot. Other types of “onsets” that ALS patients might experience, is “bulbar onset” and “respiratory onset.” Bulbar onset ALS patients have a difficulty time speaking clearly or swallowing. Their words might come out slurred, nasal in character, or quieter than usual. Respiratory onset is when the muscles that are involved with breathing are affected first. Even though these are all separate onsets, as time goes on all of them are affected, it’s just a matter of which one is affected first. However, the progression of the disease tends to be slower in patients who are younger than 40 years of age, mildly obese, have disease restricted primarily to one limb, and those with primarily upper motor neuron symptoms. In the late stages of ALS, patients experience greater difficulty with chewing and swallowing which increases the risk of them choking or inhaling food particles into the lungs. Often times, patients need to use a feeding tube to maintain a healthy weight and avoid problems such as getting aspiration pneumonia. There are other machines that help support the respiratory muscles as they weaken, such as a bilevel positive airway pressure (BPAP). However, even though this machine eases problems with breathing, it does not affect the progression of ALS. Most people that have ALS die from respiratory failure, between three to five years from the onset of symptoms.