It affects more males than females. There are several types of MND. Doctors classify them according to whether they are hereditary or not, and which neurons they affect. The types include:. Motor neurons instruct the muscles to move by passing on signals from the brain.
They play a role in both conscious and automatic movements, such as swallowing and breathing. The exact causes are unclear, but the National Institute of Neurological Diseases and Stroke reports that genetic, toxic, viral, and other environmental factors may play a role. The different types of MND cause similar symptoms and have three stages: early, middle, and advanced. The diseases progress at different speeds and vary in severity. In the early stage of MND, symptoms develop slowly and can resemble those of other health conditions.
The specific symptoms depend on the type of MND and the area of the body it affects. Typical symptoms begin in one of the following areas:. A study suggests that up to half of people with ALS experience brain involvement, including memory and language problems.
Some people also experience insomnia , anxiety , and depression. Eventually, a person with advanced ALS needs help moving, eating, breathing, or a combination of these. The disease can become life threatening, and breathing problems are the most common cause of death. MND can develop in adults or children, depending on the type. The serum Venereal Disease Research Laboratories VDRL test, erythrocyte sedimentation rate, and measurement of certain antibodies rheumatoid factor, Lyme titer, HIV, hepatitis C virus, antinuclear [ANA], anti-Hu [to check for anti-Hu paraneoplastic syndrome] are indicated only if suggested by risk factors or history.
Genetic testing eg, for superoxide dismutase gene mutation or genetic abnormalities that cause spinal muscular atrophies and enzyme measurements eg, hexosaminidase A for Tay-Sachs disease should not be done unless patients are interested in genetic counseling; disorders detected by these tests have no known specific treatments.
The mainstay of care for patients with amyotrophic lateral sclerosis is timely intervention to manage symptoms. No drug offers a substantial clinical benefit for patients with ALS.
However, riluzole may provide limited improvement in survival by 2 to 3 months , and edaravone may slow the decline in function to some degree. For cramps, quinine or phenytoin. To decrease saliva production, a strong anticholinergic drug eg, glycopyrrolate , amitriptyline , benztropine , trihexyphenidyl , transdermal hyoscine, atropine.
For pseudobulbar affect, amitriptyline , fluvoxamine , or a combination of dextromethorphan and quinidine. In patients with progressive bulbar palsy, surgery to improve swallowing has had limited success. Suspect ALS in patients with upper and lower motor neuron signs plus weakness in facial muscles. The mainstay of treatment is supportive measures eg, multidisciplinary support to help cope with disability; drug treatment for symptoms such as spasticity, cramps, and pseudobulbar affect.
In patients with ALS, riluzole may provide limited survival benefit, and edaravone may slow the decline in function. From developing new therapies that treat and prevent disease to helping people in need, we are committed to improving health and well-being around the world.
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More Content. Click here for Patient Education. Electrodiagnostic tests. Other disorders that cause pure muscle weakness should be ruled out:. Know someone who deserves recognition? Submit a nomination form here. Search this website. Sign up. There are two groups of motor neurons - upper motor neurons travelling from the brain down the spinal cord, and lower motor neurons branching outwards to supply muscles in the face, throat, arms, chest and legs.
Both groups of neurons tend to be involved in MND but to varying extents, which is one of the many reasons why each patient's disease is subtly unique. MND was first described in the mid-late s, and the French Neurologist Jean-Martin Charcot is widely credited with the first detailed descriptions. He noticed that a common feature of most cases was muscle wasting, the medical term for which is amyotrophy literally 'lack of muscle growth'. This is a feature of degeneration of lower motor neurons.
The resulting muscle wasting can be thought of as a wilting leaf when the water supply from a damaged branch fails - there is nothing intrinsically wrong with the muscle but it wastes when there is no electrical or 'nutritional' input from the lower motor neuron.
When Charcot went on to look at the spinal cords of patients with MND, he also noticed scarring of the descending upper motor neuron pathways from the brain. The medical term for this is lateral sclerosis literally scarring of the outermost pathways of the cord.
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