What is motor neuron disease?

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What are the causes? How does it occur? What will be the effects? How it can be cured?
The motor neuron diseases (MND) are a group of progressive neurological disorders that destroy motor neurons, the cells that control voluntary muscle activity such as speaking, walking, breathing, and swallowing. Characteristic symptoms of MND include gradual weakening, wasting away, and uncontrollable twitching of the muscles; spasticity or stiffness in the arms and legs; and overactive tendon reflexes. Sensation, intellect, memory, and personality are not affected in MND. In some types of MND, such as amyotrophic lateral sclerosis (ALS, commonly called Lou Gehrig’s disease) muscle weakness is progressive and eventually leads to death when the muscles that control breathing no longer work. Other types of MND progress slowly and last over a lifetime. MND occur in adults and children, and are more common in men than in women. In adults, symptoms usually appear after age 40, and may be similar to those of other diseases, making diagnosis difficult. In children, particularly in inherited forms of the disease, symptoms are present from birth. Inherited forms of MND are caused by genetic mutations or deletions that cause degeneration of motor neurons. Hereditary MND include a group of childhood disorders known as the spinal muscular atrophies. Nonhereditary (also called sporadic) MND are caused by unknown factors, although scientists think environmental toxins or viruses could act as disease triggers. Nonhereditary MND include ALS, progressive bulbar palsy, pseudobulbar palsy, primary lateral sclerosis, progressive muscular atrophy, and post-polio syndrome. There are no specific tests to diagnose the MND.  (+ info)

can any1 plz tell me the cure for "motor neuron disease" and the treatment options?

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im asking on behalf of a female patient,age 36,married.she developed this disease when she got pregnant 2yrs ago and is still suffering from it.plz plz if any1 can help.i will be grateful.thanks
thank u all for the answers below..my friend is taking riluzole n she feels better with it but im scared about its side effects.
also can u tell me how the differential diagnosis for ALS is made becoz the doc just did the EMG test n said that its ALS.
Hello..I am sorry to hear this news..I am not to aware of the pathology with NMD, However I did find this informative explanation on the Wikipedia site. Please click on the link I have given you below and this should answer some of your questions in short but informative detail.
Best Wishes to you!
George  (+ info)

what causes motor neuron disease?

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I have listed all articles with the links below. I found the answer stating Vitamin E deficiency interesting. It looks like there is no definite cause though. Good luck and God Bless!


Mutation in a gene that codes for a protein involved in transporting materials within nerve cells causes muscle weakness and wasting, according to a report in the April issue of Nature Genetics. The findings could help hone in on the causes of related diseases.
If motor neurons - nerves cells along which the brain sends instructions to muscles - become damaged, muscles start to weaken and eventually stop working. By studying a family with individuals suffering from an inherited type of motor neuron disease that affects the vocal cords and muscles of the face, hands and feet, Imke Puls and colleagues at the National Institutes of Health, have identified the genetic cause of the disease. The scientists found a mutation in a gene that codes for a component of dynactin, part of the molecular "engine" that shunts materials, including nutrients required for proper functioning and survival of nerve cells, along "tracks" within the nerve cells. The mutation reduces the ability of dynactin to bind to these tracks - called microtubules - thereby disrupting transport within nerve cells and eventually causing the cells to degenerate.

Mutations in other proteins involved in transport within nerve cells may underlie other types of motor neuron disease.

Author contact:

Imke Puls
National Institute of Neurological Disorder and Stroke
NIH, Bethesda
MD, USA
Tel: +1 301 435 9288
E-mail: [email protected]

http://hum-molgen.org/NewsGen/03-2003/msg10.html

Causes
About 90% of cases of MND are "sporadic", meaning that the patient has no family history of ALS and the case appears to have occurred with no known cause. Genetic factors are suspected to be important in determining an individual's susceptibility to disease, and there is some weak evidence to suggest that onset can be "triggered" by as yet unknown environmental factors (see 'Epidemiology' below).

Approximately 10% of cases are "familial MND", defined either by a family history of MND or by testing positive for a known genetic mutation associated with the disease. The following genes are known to be linked to ALS: Cu/Zn superoxide dismutase SOD1, ALS2, NEFH(a small number of cases), senataxin (SETX) and vesicle associated protein B (VAPB).

Of these, SOD1 mutations account for some 20% of familial MND cases. The SOD1 gene codes for the enzyme superoxide dismutase, a free radical scavenger that reduces the oxidative stress of cells throughout the body. So far over 100 different mutations in the SOD1 gene have been found, all of which cause some form of ALS(ALSOD database). In North America, the most commonly occurring mutation is known as A4V and occurs in up to 50% of SOD1 cases. In people of Scandinavian extraction there is a relatively benign mutation called D90A which is associated with a slow progression. Future research is concentrating on identifying new genetic mutations and the clinical syndrome associated with them. Familial MND may also confer a higher risk of developing cognitive changes such as frontotemporal dementia or executive dysfunction (see 'extra-motor change in MND' below).

It is thought that SOD1 mutations confer a toxic gain, rather than a loss, of function to the enzyme. SOD1 mutations may increase the propensity for the enzyme to form protein aggregates which are toxic to nerve cells.

http://en.wikipedia.org/wiki/Motor_neuron_disease#Causes

It seems there are several answers to this although the one below is interesting:

Through a series of clinical studies and observations the past 10 years, researchers at Cornell University in New York have discovered that a vitamin E deficiency is the cause of equine motor neuron disease (EMND). This is a rare neurodegenerative disorder of the somatic lower motor neurons, which are found in the spinal cord, writes Sarah E. Hogwood.

http://www.jupiterhorsemens.org/motor_neuron_disease.htm  (+ info)

does any one know of Motor Neuron Disease ?

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It is a progressive terrible illness, the brain remains in tact but everything else deteriorates. I watched a good friend suffer with this disease, she was only fourty when she was diagnosed which is no age. Good luck to you and your family.  (+ info)

Physiotherapy management for Motor Neuron Disease.?

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Respiratory management for MND.
Physiotherapy management for MND.
Physical exercise can help maintain or improve strength in the muscles not affected by MND, and maintain flexibility in muscles which are affected. It can help prevent stiffness in the joints. Physiotherapy may also help people with breathing difficulties to clear their chests and maintain lung capacity. However, people living with MND can tire very easily and find they need to conserve energy, so very strenuous exercise is not normally recommended.
Do try this link to get to know how other people with MND have found exercise routines helpful.
http://www.healthtalkonline.org/Nerves_and_brain/motorneuronedisease/Topic/3434/  (+ info)

Question about Motor Neuron Disease?

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My Grandfather died of MND 6 years ago. I'm really worried it is hereditary. How do you know if it is. I'm only 15 but still worried for my mum's sake and my siblings sake. Thank you
You and your family can be tested for genetic MND.  (+ info)

Do you know of anyone with Motor Neuron Disease?

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I am wondering from a personal perspective if there are ant stories of things that arnt really tested even or miricales of people being found to have a greater quality of life or improvements that may have even been discounted. I know all about the "no cure" status and the drug that helps a bit I cant remember its name and I know a lot about the disease also......etc. My grandmother had it.
thanks starligh I was thinking about what you said about stephn hawkns also although he i believe has a type I believe that can lead to a longer survival rate
Unfortunately, there have really been no developments in treatment for it. The disease itself is still not fully understood, and attempts to make improvements through drugs, etc. have largely failed. I'm sure if there were truly any treatment at all that would help, Stephen Hawking would have tried it by now.  (+ info)

weak arm muscles must it mean motor neuron disease?

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There are many reason why someone has weak arm muscles. Do you work out and lift things - The more your work the muscle - the stronger it will get. Also you may not taking in enough protein to build enough muscle to do whatever you need to do.  (+ info)

is motor neuron disease hereditary ?

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About 90% of cases of MND are "sporadic", meaning that the patient has no family history of ALS and the case appears to have occurred with no known cause. Genetic factors are suspected to be important in determining an individual's susceptibility to disease, and there is some weak evidence to suggest that onset can be "triggered" by as yet unknown environmental factors (see 'Epidemiology' below).

Approximately 10% of cases are "familial MND", defined either by a family history of MND or by testing positive for a known genetic mutation associated with the disease. The following genes are known to be linked to ALS: Cu/Zn superoxide dismutase SOD1, ALS2, NEFH (a small number of cases), senataxin (SETX) and vesicle associated protein B (VAPB).  (+ info)

motor neuron disease?

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affects 1 in ____ people?

please source your findings.
Try doing a search...
http://www.ninds.nih.gov/disorders/motor_neuron_diseases/detail_motor_neuron_diseases.htm  (+ info)

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