ALS affects both your upper and lower motor neurons. With ALS, you gradually lose control over the muscles that help you walk, talk, chew, swallow, and breathe. Over time, they weaken and waste away. You may also have stiffness and twitches in your muscles.
ALS usually starts between the ages of 40 and 60. Most people with the disease live for 3 to 5 years after their symptoms start, yet some people can live for 10 years or longer.
Primary lateral sclerosis (PLS):
PLS is similar to ALS, but it affects only upper motor neurons.
It causes weakness and stiffness in the arms and legs, a slowed walk, and poor coordination and balance. The speech also becomes slow and slurred.
Like ALS, it usually starts in people 40 to 60 years old. The muscles get stiffer and weaker over time. But unlike ALS, people don't die from it.
Progressive Bulbar palsy(PBP):
This is a form of ALS, many people with this condition will eventually develop ALS. PBP damages motor neurons in the brain stem, which is at the base of your brain. The stem has motor neurons that help you chew, swallow, and speak. With PBP, you might slur words and have trouble chewing and swallowing. It also makes it hard to control emotions. You may laugh or cry without meaning to.
Pseudobulbar palsy:
This is similar to progressive bulbar palsy. It affects motor neurons that control the ability to talk, chew, and swallow. Pseudobulbar palsy causes people to laugh or cry with no control.
Progressive muscular atrophy:
This form is much less common than ALS or PBP. It can be inherited or sporadic. Progressive muscular atrophy mainly affected your lower motor neurons. Weakness usually starts in your hands and then spreads to other parts of the body. muscles get weak and may cramp. This disease can turn into ALS.
Spinal muscular atrophy:
This is an inherited condition that affects lower motor neurons. A defect in a gene called SMN1 causes spinal muscular atrophy. This gene makes a protein that protects your motor neurons. Without it, they die. This causes weakness in the upper legs and arms, and in the trunk.
SMA comes in different types that are based on when symptoms first appear:
Type 1: It starts around age 6 months. Children with this type can't sit on their own or hold up their heads. They have weak muscle tone, poor reflexes, and trouble swallowing and breathing.
Type 2: It starts between 6 and 12 months. Kids with this form can sit, but they can't stand or walk alone. They may also have trouble breathing.
Type 3: It starts between the ages of 2 and 17. It affects how a child can walk, run, stand up, and climb stairs. Kids with this type may also have a curved spine or shortened muscles or tendons or tendons around their joints.
Type 4: It usually starts after age 30. People with this type may have muscle weakness, shaking twitching, or breathing problems. It mainly affects muscles in the upper arms and legs.
Kennedy's disease:
It's also inherited, and it affects only males. Females can be carriers but don't get ill from it. A woman with Kennedy's disease gene has a 50% chance of passing it on to a son. Males with Kennedy's disease have shaken hands, muscle cramps and twitches, and weakness in their faces, arms, and legs. They may have trouble swallowing and speaking. Men can have enlarged breasts and a low sperm count.
Sign and symptoms:
Motor neuron diseases typically manifest as a group of movement-related symptoms. They come on slowly and worsen over more than three months. Various patterns of muscle weakness are seen, and muscle cramps and spasms may occur.
- One can have difficulty breathing with climbing stairs.
- difficulty breathing when lying down
- Respiratory failure if breathing muscles become involved.
- Bulbar symptoms, including difficulty speaking
- Difficulty in swallowing
- excessive saliva production
Motor neuron disease is seen both in children and in adults. Those that affect children tend to be inherited or familial, and their symptoms are either present at birth or appear before learning to walk. Those that affect adults tend to appear after age 40. The clinical course depends on the specific disease, but most progress or worsens over the course of months.
Causes of motor neuron disease:
Motor neuron disease occurs when specialist nerve cells in the brain and spinal cord progressively lose their function. In most cases, motor neuron disease won't have a family history of the condition. This is known as sporadic motor neuron disease. As we get older, we may gradually lose the ability to keep this damage under control, triggering irreversible neurodegeneration.
It's still unclear whey the motor neurons begin to lose function. Most experts believe that it's a combination of interrelated factors that ultimately affect either the motor neurons or the nerve cells that support them.
Aggregates and RNA processing:
Aggregates are abnormal clumps of protein that develop inside motor neurons. They are found in nearly all cases of motor neurons disease and may disrupt the normal working of the motor neurons, or at least be a marker that the cells are under great strain.
Cell transport disruption:
All cells contain transport system,s that nutrients and other chemicals into the cell and waste products out of the cell. Research suggests that the transport systems in motor neurons become disrupted. Over time, toxic waste can build up in cells as a natural by-product of normal cell activity.
The body gets rid of the toxic waste by producing substances known as antioxidants, and packaging the waste into containers called microvesicles. Research suggests that in motor neuron disease the motor neurons may be deficient in antioxidants. However, there's no evidence that this is due to poor dietary intake.
Glial cells:
Glia is cells that surround and support motor neurons and provide them with nutrients. Glial cells also help relay information from one nerve cell to another. Some cases of motor neuron disease may be caused by problems with the glial cells which means that the motor neurons no longer receive the support and nutrition they need to function normally.
Glutamate:
Nerve cells use special "Messenger chemicals" called neurotransmitters to pass information from one cell to another. One of the neurotransmitters is called glutamate. There's evidence that the motor neurons in people with motor neuron disease may have become more sensitive to glutamate, resulting in damage to these cells. However, this isn't linked to dietary intake of glutamate.
Mitochondria:
Mitochondria are the "batteries" of cells. They provide the energy that a cell needs to carry out its normal functions. Research has shown that the mitochondria in the motor neurons of people with motor neuron disease seem to become abnormal.
Diagnosis:
After an initial examination, they may refer to a brain and nervous system specialist for further opinion and possibly tests. Diagnosing motor neuron disease can be difficult during the condition's initial stages because many of the early symptoms can be caused by other more common health conditions such as:
Trapped nerve wear and tear of the bones in the spine can sometimes cause nerves to become trapped and compressed under the spine
- Peripheral neuropathy- where part of the nervous system becomes damaged by another health condition like type 2 diabetes.
Motor neuron disease is usually diagnosed by a neurologist based on the symptoms and a physical examination.
There's no single test for motor neuron disease, but the diagnosis is often obvious from a person's symptoms and physical examination by a neurologist. Various tests may be used to rule out other possible causes ft the person's symptoms if they seem likely, include:
- Blood test: checking that certain organs, such as your thyroid, are working normally, or to look for a marker of muscle weakness
- Magnetic resonance imaging: Detailed image of the inside of the brain and spinal cord.
- Electromyography: Measures the electrical activity in your muscles, which shows how well your motor neurons are working and connecting to the muscles.
- Nerve conduction test: Similar to an EMG but measures how quickly your nerves can conduct an electrical single.
- Lumbar puncture: A sample of spinal fluid may be removed for testing to exclude an inflammatory nerve condition.
- Muscle biopsy: Rare cases, a small pf muscle may be removed for testing to determine whether the problem lies in the muscles or nerves.
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