5/18/2023 0 Comments Als emg findingsLimb-onset ALS begins with weakness in the arms and legs and accounts for about two-thirds of all classic ALS cases. Ĭlassic ALS accounts for about 70% of all cases of ALS and can be subdivided into limb-onset ALS (also known as spinal-onset) and bulbar-onset ALS. There is debate over whether PLS and PMA are separate diseases or simply variants of ALS. Primary lateral sclerosis (PLS) involves only upper motor neurons, and progressive muscular atrophy (PMA) involves only lower motor neurons. Typical or "classical" ALS involves upper motor neurons in the brain and lower motor neurons in the spinal cord. ĪLS can be classified by the types of motor neurons that are affected. Typical or "classical" ALS involves neurons in the brain ( upper motor neurons) and in the spinal cord ( lower motor neurons). A few people with ALS have symptoms that are limited to one spinal cord region for at least 12 to 24 months before spreading to a second region these regional variants of ALS are associated with a better prognosis. In most cases the disease spreads and affects other spinal cord regions. In about 5% of cases, muscles in the trunk of the body are affected first. In about 25% of cases, muscles in the face, mouth, and throat are affected first because motor neurons in the part of the brainstem called the medulla oblongata (formerly called the "bulb") start to die first along with lower motor neurons. ĪLS itself can be classified in a few different ways: by how fast the disease progresses by whether it is familial or sporadic, and by the region first affected. 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% survive longer than ten years. The disease can affect people of any age, but usually starts around the age of 60. Mechanical ventilation can prolong survival but does not stop disease progression, with death usually caused by respiratory failure. 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. 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. Motor neuron loss continues until the ability to eat, speak, move, and finally the ability to breathe is lost with the cause of early death usually being respiratory failure. 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 speaking or swallowing. Early symptoms of ALS include stiff muscles, muscle twitches, and gradual increasing weakness and muscle wasting. ALS is the most common form of the motor neuron diseases. Suspected as based on symptoms and supported by MRI Īmyotrophic lateral sclerosis ( ALS), also known as motor neuron 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 These cause symptoms in skeletal muscles throughout the body.Įarly: Stiff muscles, muscle twitches, gradual increasing weakness The parts of the nervous system affected by ALS, highlighting the upper motor neurons, lower motor neurons, and frontotemporal regions of the brain.
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