ALS
Rare neurodegenerative disease / From Wikipedia, the free encyclopedia
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Amyotrophic lateral sclerosis (ALS), also known as motor neurone disease (MND) or Lou Gehrig's disease in the United States, is a rare, terminal neurodegenerative disorder that results in the progressive loss of both upper and lower motor neurons that normally control voluntary muscle contraction.[3] ALS is the most common form of the motor neuron diseases.[8] ALS often presents in its early stages with gradual muscle stiffness, twitches, weakness, and wasting.[3] Motor neuron loss typically continues until the abilities to eat, speak, move, and, lastly, breathe are all lost.[3] While only 15% of people with ALS also fully develop frontotemporal dementia, an estimated 50% face at least some minor difficulties with thinking and behavior.[9] Depending on which of the aforementioned symptoms develops first, ALS is classified as limb-onset (begins with weakness in the arms or legs) or bulbar-onset (begins with difficulty in speaking or swallowing).[10]
Most cases of ALS (about 90–95%) have no known cause, and are known as sporadic ALS.[3][11] However, both genetic and environmental factors are believed to be involved.[12] The remaining 5–10% of cases have a genetic cause, often linked to a history of the disease in the family, and these are known as familial ALS (hereditary).[6][13] About half of these genetic cases are due to disease-causing variants in one of four specific genes.[14] The diagnosis is based on a person's signs and symptoms, with testing conducted to rule out other potential causes.[3]
There is no known cure for ALS.[3] The goal of treatment is to slow the disease progression, and improve symptoms.[9] Treatments that slow ALS include riluzole (extends life by two to three months) and sodium phenylbutyrate/ursodoxicoltaurine (extends life by around seven months).[15][16] Non-invasive ventilation may result in both improved quality, and length of life.[5] Mechanical ventilation can prolong survival but does not stop disease progression.[17] A feeding tube may help maintain weight and nutrition.[18] Death is usually caused by respiratory failure.[19] The disease can affect people of any age, but usually starts around the age of 60.[19] The average survival from onset to death is two to four years, though this can vary, and about 10% of those affected survive longer than ten years.[20]
Descriptions of the disease date back to at least 1824 by Charles Bell.[21] In 1869, the connection between the symptoms and the underlying neurological problems was first described by French neurologist Jean-Martin Charcot, who in 1874 began using the term amyotrophic lateral sclerosis.[21]