Motor Neuron Disease
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Motor Neuron Disease

Motor neuron disease (MNDS) is a progressive, degenerative disorder that affects nerves in the upper or lower parts of the body. Most of the time motor neuron diseases are acquired, but approximately five to ten per cent of the time the disease is hereditary. The following diseases are classified as a motor neuron disease:

  • Amyotrophic lateral sclerosis (ALS or Lou Gehrig's Disease)
  • Progressive muscular atrophy
  • Postpolio syndrome

Motor neuron disease usually occurs in people between the ages of 40-70 with the average at 55. There have been a few cases where onset has started as early as in the teens. The diagnosis of MND is made from a physical examination, an EMG study and a pattern of finding and excluding other diseases which allows the neurologist to establish a diagnosis and to determine which form of the disease is present.

Unfortunately, there is not a specific test available which gives a "yes" or "no" answer. Symptoms may include difficulty swallowing, limb weakness, slurred speech, impaired gait, facial weakness, and muscle cramps. Respiration problems may occur but this usually happens in the later stages of the disease.

The cause(s) of most MNDs are unknown, but environmental, toxic, viral, or genetic factors may be implicated. Currently, treatment options include, physical therapy, drug therapy, and assistive devices may help some people. The prognosis for people living with a MND varies depending on the type, for some—such as people with ALS—the outcome is terminal.

At this time there is no cure for motor neuron disease. There is one medication available by prescription —Rilutek— which has shown in clinical trials to slow the progression of ALS slightly. Over the last ten years it appears that people with MNDs are living longer because of improved care and increased knowledge of the disease.

ALS Research News - The latest ALS als research articles.
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3.  Genetic contributors to motor neuron diseases

The recent discovery of a number of genes known to cause motor neuron diseases such as ALS has provided new insights into the mechanisms underlying motor neuron degeneration, according to a recent art ◊

"Genetic contributors to motor neuron diseases"
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At the 20th Internal Symposium on ALS/MND, researchers presented the results of a Phase 2 clinical trial testing the safety and tolerability of a drug called KNS-760704 for use by ALS patients. KNS-7 ◊

"Encouraging trial results for KNS-760704 "
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8.  TDP-43 mutations cause motor deficits in zebrafish

Mutations in the TAR DNA-binding protein (TDP-43) gene cause motor neuron defects in zebrafish and lead to impaired swimming, researchers from Université de Montreal report. Interestingly, insufficie ◊

"TDP-43 mutations cause motor deficits in zebrafish"