Motor neuron diseases destroy cells responsible for controlling voluntary muscle actions, causing difficulty in walking, breathing, swallowing, and speaking. There are several types, including ALS, which affects men more due to X-linked genes. Symptoms include muscle weakness and wasting, difficulty swallowing, speaking, and breathing. There is no cure, but physical therapy and medication can help manage symptoms.
Motor neuron diseases are progressive disorders that affect the neurological system by destroying cells called motor neurons. These cells are responsible for controlling voluntary muscle actions. People with motor neuron disease have difficulty controlling actions such as walking, breathing, swallowing, and speaking. There are several types of motor neuron disease, including primary lateral sclerosis, spinal muscular atrophy, and amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease. The terms “motor neuron disease” and “amyotrophic lateral sclerosis” are often used interchangeably, particularly in the United States and Canada.
Motor neuron diseases occur in both children and adults and can occur in both genders, although they are more common in men. This is because some types of motor neuron disease are X-linked, which means that the gene that causes the disease is on the X chromosome. A woman can inherit a defective copy of the gene without developing the disease, because she has two X chromosomes and so he also has a normal copy of the gene. Conversely, if a man inherits a defective copy of the gene, there is no second X chromosome to provide a normal copy.
In classic ALS, the first symptoms of motor neuron disease that appear are usually related to the function of the hands, arms, or legs. Muscle weakness and wasting are common initial symptoms. Additionally, individuals may have difficulty swallowing. As the disease progresses, the muscles become increasingly weak and more muscle groups are affected. In addition to difficulty swallowing, it is common for an individual to begin having difficulty speaking and breathing, due to weakness and wasting of the diaphragm and chest muscles. Motor neuron diseases rarely impair cognitive function.
Other types of motor neuron disease cause slightly different symptoms. In primary lateral sclerosis, for example, the legs, arms and hands are most affected, and individuals experience problems with balance, muscle stiffness and weakness. In spinal muscular atrophy, the muscle weakness and wasting is most severe in the legs, and individuals do not always experience loss of control of breathing, speaking, or swallowing.
Motor neuron diseases cannot be cured and there are no standard treatment protocols for these diseases. In most cases, treatment for motor neuron disease includes physical therapy, medications to treat symptoms, and other supportive treatments. For example, medications are often prescribed to relieve pain, relax muscles, and relieve inflammation. Physical therapy is used to preserve muscle tone, strength and flexibility and to slow the rate of muscle wasting. Occupational therapy and devices such as a wheelchair or speech synthesizer help the individual maintain as much independence as possible.
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