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Amyotrophic lateral sclerosis is a progressive disease that affects motor neurons, which are specialized nerve cells that control muscle mobility. Most people with ALS have a sporadic disease, meaning they occur in people with no apparent history of the disease in their family. People with sporadic ALS typically first show signs of the disorder in their late fifties or early sixties. A small number of people with ALS have a family history with ALS or a related condition called frontotemporal dementia, which is a progressive brain disorder that affects personality, habits, and language. ALS signs and symptoms first appear in one's late forties or early fifties. People with familial ALS have symptoms in childhood or teens, but not so often. The first signs and symptoms of ALS may be so subtle that they are skipped over. Many people with ALS experience hunger as a result of reduced food intake due to dysphagia and an increase in their body's energy needs as a result of prolonged illness. Muscles get weaker as the disease progresses, and arms and legs begin to look thinner as muscle tissue atrophies. ALS sufferers die from respiratory failure within 2 to ten years after the signs and symptoms of ALS first appeared; however, disease progression varies among affected individuals. FTD accounts for about 20% of people with ALS.
Muscle weakness can also impact respiratory and heart muscles, resulting in life-threatening breathing difficulties and heart failure. About half of all adults with IBMPFD have a condition called Paget disease of bone. Bone loss affects the hips, spine, and skull, as well as long bones of the arms and legs, which is the most common condition. Other aspects of Paget disease of bone decay vary depending on which bones are affected. Risefully, bone loss can weaken bones enough that they break. IBMPFD affects the brain in about one-third of people with IBMPFD. People with frontotemporal dementia may have a difficult time speaking, recalling words and names, and using numbers. People with IBMPFD typically live into their fifties or sixties.
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