Amyotrophic lateral sclerosis | |
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Other names |
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Parts of the nervous system affected by ALS, causing progressive symptoms in skeletal muscles throughout the body[2] | |
Specialty | Neurology |
Symptoms | Early: Stiff muscles, muscle twitches, gradual increasing weakness[3] Later: Difficulty in speaking, swallowing, and breathing; respiratory failure;[3] 10–15% experience frontotemporal dementia[2] |
Complications | Falling (accident); Respiratory failure; Pneumonia; Malnutrition |
Usual onset | 45–75 years[2] |
Causes | Unknown (about 85%), genetic (about 15%) |
Risk factors | Genetic risk factors; age; male sex; heavy metals; organic chemicals; smoking; electric shock; physical exercise; head injury[2] |
Diagnostic method | Clinical diagnosis of exclusion based on progressive symptoms of upper and lower motor neuron degeneration in which no other explanation can be found. Supportive evidence from electromyography, genetic testing, and neuroimaging |
Differential diagnosis | Multifocal motor neuropathy, Kennedy's disease, Hereditary spastic paraplegia, Nerve compression syndrome, Diabetic neuropathy, Post-polio syndrome, Myasthenia gravis, Multiple sclerosis[4] |
Treatment | Walker (mobility); Wheelchair; Non-invasive ventilation;[5] Feeding tube; Augmentative and alternative communication; symptomatic management |
Medication | Riluzole, Edaravone, Sodium phenylbutyrate/ursodoxicoltaurine, Tofersen, Dextromethorphan/quinidine |
Prognosis | Life expectancy highly variable but typically 2–4 years after diagnosis[6] |
Frequency |
Amyotrophic lateral sclerosis | |
---|---|
Other names |
|
Parts of the nervous system affected by ALS, causing progressive symptoms in skeletal muscles throughout the body[2] | |
Specialty | Neurology |
Symptoms | Early: Stiff muscles, muscle twitches, gradual increasing weakness[3] Later: Difficulty in speaking, swallowing, and breathing; respiratory failure;[3] 10–15% experience frontotemporal dementia[2] |
Complications | Falling (accident); Respiratory failure; Pneumonia; Malnutrition |
Usual onset | 45–75 years[2] |
Causes | Unknown (about 85%), genetic (about 15%) |
Risk factors | Genetic risk factors; age; male sex; heavy metals; organic chemicals; smoking; electric shock; physical exercise; head injury[2] |
Diagnostic method | Clinical diagnosis of exclusion based on progressive symptoms of upper and lower motor neuron degeneration in which no other explanation can be found. Supportive evidence from electromyography, genetic testing, and neuroimaging |
Differential diagnosis | Multifocal motor neuropathy, Kennedy's disease, Hereditary spastic paraplegia, Nerve compression syndrome, Diabetic neuropathy, Post-polio syndrome, Myasthenia gravis, Multiple sclerosis[4] |
Treatment | Walker (mobility); Wheelchair; Non-invasive ventilation;[5] Feeding tube; Augmentative and alternative communication; symptomatic management |
Medication | Riluzole, Edaravone, Sodium phenylbutyrate/ursodoxicoltaurine, Tofersen, Dextromethorphan/quinidine |
Prognosis | Life expectancy highly variable but typically 2–4 years after diagnosis[6] |
Frequency |