Īs a disease, ALS itself can be classified in a few different ways: by which part of the motor neurons are affected by the parts of the body first affected whether it is genetic and the age at which it started. Other motor neuron diseases include primary lateral sclerosis (PLS), progressive muscular atrophy (PMA), progressive bulbar palsy, pseudobulbar palsy, and monomelic amyotrophy (MMA). ĪLS is a motor neuron disease, which is a group of neurological disorders that selectively affect motor neurons, the cells that control voluntary muscles of the body. 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. ĭescriptions of the disease date back to at least 1824 by Charles Bell. 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. The disease can affect people of any age, but usually starts around the age of 60. Death is usually caused by respiratory failure. A feeding tube may help maintain weight and nutrition. Mechanical ventilation can prolong survival but does not stop disease progression. Non-invasive ventilation may result in both improved quality, and length of life. Treatments that slow ALS include riluzole (extends life by two to three months) and sodium phenylbutyrate/ursodoxicoltaurine (extends life by around seven months). The goal of treatment is to slow the disease progression, and improve symptoms. The diagnosis is based on a person's signs and symptoms, with testing conducted to rule out other potential causes. About half of these genetic cases are due to disease-causing variants in one of four specific genes. The remaining 5% to 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). 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. Motor neuron loss continues until the abilities to eat, speak, move, or, lastly, breathe are lost. Around half of 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 in speaking or swallowing. Early symptoms of ALS include stiff muscles, muscle twitches, gradual increasing weakness, and muscle wasting. ALS is the most common form of the motor neuron diseases. Incidence: 1.6/100,000 individuals per year Prevalence: 4.4/100,000 living individuals Īmyotrophic lateral sclerosis ( ALS), also known as motor neurone disease ( MND) or Lou Gehrig's disease, is a rare neurodegenerative disease that results in the progressive loss of motor neurons that control voluntary muscles. Life expectancy highly variable but typically 2–4 years after diagnosis Riluzole, Edaravone, Sodium phenylbutyrate/ursodoxicoltaurine, Tofersen, Dextromethorphan/quinidine Walker (mobility) Wheelchair Non-invasive ventilation Feeding tube Augmentative and alternative communication symptomatic management Multifocal motor neuropathy, Kennedy's disease, Hereditary spastic paraplegia, Nerve compression syndrome, Diabetic neuropathy, Post-polio syndrome, Myasthenia gravis, Multiple sclerosis Supportive evidence from electromyography, genetic testing, and neuroimaging Genetic risk factors age male sex heavy metals organic chemicals smoking electric shock physical exercise head injury Ĭlinical diagnosis of exclusion based on progressive symptoms of upper and lower motor neuron degeneration in which no other explanation can be found. Later: Difficulty in speaking, swallowing, and breathing respiratory failure 10-15% experience frontotemporal dementia įalling (accident) Respiratory failure Pneumonia Malnutrition Parts of the nervous system affected by ALS, causing progressive symptoms in skeletal muscles throughout the body Įarly: Stiff muscles, muscle twitches, gradual increasing weakness,
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