The disease can affect people of any age, but usually starts around the age of 60 and in inherited cases around the age of 50. Mechanical ventilation can prolong survival but does not stop disease progression. Non-invasive ventilation may result in both improved quality and length of life. A medication called riluzole may extend life by about two to three months. The goal of treatment is to improve symptoms. The diagnosis is based on a person's signs and symptoms, with testing done to rule out other potential causes. The underlying mechanism involves damage to both upper and lower motor neurons in ALS-FTD, neurons in the frontal and temporal lobes of the brain die as well.
ALS and frontotemporal dementia (FTD) are considered to be part of a common disease spectrum (ALS-FTD) because of genetic, clinical, and pathological similarities. About half of these genetic cases are due to one of two specific genes. The remaining 5% to 10% of cases have a genetic cause linked to a history of the disease in the family, and these are known as familial ALS. However, both genetic and environmental factors are believed to be involved. Most cases of ALS (about 90% to 95%) have no known cause, and are known as sporadic ALS. ALS eventually causes paralysis and early death, usually from respiratory failure. Motor neuron loss continues until the ability to eat, speak, move, and finally the ability to breathe is lost.
The affected muscles are responsible for chewing food, speaking, and walking. Half of the people with ALS develop at least mild difficulties with thinking and behavior, and about 15% develop frontotemporal dementia. Limb-onset ALS begins with weakness in the arms or legs, while bulbar-onset ALS begins with difficulty speaking or swallowing. Early symptoms of ALS include stiff muscles, muscle twitches, and gradual increasing weakness and muscle wasting. ALS is the most common type of motor neuron disease. Suspected as based on symptoms and supported by MRI Īmyotrophic lateral sclerosis ( ALS), also known as motor neurone disease ( MND) or Lou Gehrig's disease, is a neurodegenerative disease that results in the progressive loss of motor neurons that control voluntary muscles. Later: Difficulty in speaking, swallowing, and breathing respiratory failure Medical condition Amyotrophic lateral sclerosisĪn MRI of the brain with increased T2 signal in the posterior part of the internal capsule that can be tracked to the motor cortex, consistent with the diagnosis of ALSĮarly: Stiff muscles, muscle twitches, gradual increasing weakness
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