Motor Neurone Disease strikes about 5 out of every 100,000 people in the UK, usually occurring between the ages of 50 and 70, and affects men more than women. From the time we are first born, our brain sends directions to the various parts of our body. These signals or instructions are sent as electrical impulses and control everyday functions like walking, talking, and swallowing. As the infant grows more neurons are formed based on how much stimulation they experience in their environment, which is why pediatricians and other child experts suggest parents actively engage with their babies from the earliest ages. Motor neurons deal mainly with the motor functions such as walking and running, moving our arms and hands, and other body movements. In Motor Neurone Disease the nerve cells that control these motor functions are destroyed. Without regular movement the muscles can atrophy and eventually die.
There are three main types of Motor Neurone Disease. The first is called ALS or Amyotrophic lateral sclerosis, which results in muscle stiffness and weakness. The second type is PMA, or Progressive muscle atrophy, which results in the wasting and weakness of the muscles, as well as uncontrolled twitching. The third type of Motor Neurone Disease is called Progressive bulbar palsy, and causes the person to have difficulty speaking and swallowing. Of these three types of Motor Neurone Disease, ALS is the most common and found in about half the people with Motor Neurone Disease. The other two each affect about a quarter of patients with Motor Neurone Disease. There is no known reason why a person develops a Motor Neurone Disease and only a few affected people have a family history of the disease. The symptoms of Motor Neurone Disease can mimic those of other diseases. They also affect people at different rates, so one person may have symptoms that develop slowly over years while another seems to have a rush of symptoms all at once.
The first hint that there is a problem may be a suddenly clumsiness, especially with the hands, together with a general feeling of fatigue. Next a person may experience pain, muscle cramps, stiff joints, coughing, excessive saliva, constipation, choking sensation or breathing problems, and depression. As the Motor Neurone Disease advances the muscles may feel weaker and the person can not do everyday tasks. The throat muscles may continue to become even tighter, leading to more breathing problems and difficulty swallowing. While the Motor Neurone Disease does not necessarily affect the intellect or thinking parts of the brain, a few people do seem to lose some mental capacity or have a personality change. There is no one test for Motor Neurone Disease and a GP or neurologist may make the initial diagnosis based on the patients change in movement and behavior. They can also conduct tests such as an EMG (electromyelogram) to test electrical activity of muscles, or a TMS (transcranial magnetic stimulation) which measures nerve activity. Nerve conduction tests and MRI screenings are also ways to test for Motor Neurone Disease. Most people in the UK who have Motor Neurone Disease today are treated with the medication Riluzole, which is not a cure but has slowed the progress of the disease in some patients. There are also medicines and treatments that can help a patient control and manage the symptoms. New treatments for Motor Neurone Disease are being researched and developed at this time including research into a protein, TDP-43, which may be part of the damaged brain neurons, as well as gene research. The hope is that eventually there will be treatments that can hopefully cure the three main types of Motor Neurone Disease.
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