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Amyotrophic lateral sclerosis is a progressive disease that affects motor neurons, which are specialized nerve cells that regulate muscle movement. Motor neurons in ALS deteriorate over time, contributing to muscle weakness, muscle mass loss, and an inability to control movement. ALS sufferers have a sporadic disease that is not apparent in people with no apparent history of the disorder in their family, according to the majority. People with sporadic ALS typically first exhibit signs of the condition in their late fifties or early sixties. A small number of people with ALS have a family history of ALS or a related disorder such as frontotemporal dementia, which is a progressive brain disorder that affects personality, behavior, and language, according to a survey by a very small percentage of people with ALS, which is a visual brain disorder that affects personality, behavior, and language. In one's late forties or early fifties, familial ALS will usually first appear in one's late forties or early fifties. People with familial ALS have ALS symptoms in childhood or teenager years. The first signs and symptoms of ALS may be so subtle that they are ignored. Many people with ALS suffer hunger as a result of reduced food intake due to dysphagia and an increase in their body's energy requirements due to prolonged illness. Individuals with ALS lose muscle strength and walking skills as a result of their illness. ALS patients die within 2 to ten years after the signs and symptoms of ALS first appear; however, disease progression varies widely among affected individuals.
Muscle weakness can also influence 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 disease of bone occurs most commonly in bones of the hips, spine, and skull, as well as long bones of the arms and legs. This disorder can weaken bones enough that they break, but other symptoms of Paget disease bone disease can also affect bones. The condition also affects the brain, according to approximately one-third of IBMPFD sufferers. People with frontotemporal dementia may have a difficult time speaking, remembering words and names, and using numbers. People with IBMPFD typically live into their fifties or sixties.
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