motor neuron disease

Chronic disease in which there is progressive degeneration of the nerve cells which instigate movement. It leads to weakness, wasting, and loss of muscle function and usually proves fatal within two to three years of onset. Motor neuron disease occurs in both familial and sporadic forms but its causes remain unclear. A gene believed to be implicated in familial cases was discovered in 1993.

Results of a US trial in 1995 showed that the drug Myotrophin, a genetically engineered version of a chemical produced in the muscles, slowed deterioration in people with MND by 25%.

The drug Riluzole (Rilutek) is sometimes used in the treatment of MND in the USA (it is unavailable in Europe). It works by inhibiting the neurotransmitter glutamate in the brain, which is over produced during the course of the disease, killing brain cells. Riluzole is of benefit to a small percentage of sufferers only and its side effects include nausea and dizziness.