Motor Neurone Disease

  • What is the difference between motor neurone disease and ALS?

    Different names for much the same thing is confusing. Motor neurone disease (MND) is a progressive disorder of tow parts of the motor (movement) control of the body. One part is the upper motor neurone (UMn) which takes the messages for movement form the brain down to the lower motor neurone (LMn) which is the nerve cell which takes the message from the spinal cord to the muscle. The A in ALs is for amyotrophic which is a fancy name for the wasting (thinning or loss of bulk) of muscle and is cause by loss of the LMn. The LS of ALS is for lateral sclerosis, which is the change in the UMn in MND which causes weakness and spasticity. In the USA, ALS is more frequently used while we use MND - it is the same disease.

  • I have heard that Motor Neurone Disease can be passed on from family who have Alzheimer's disease. Is this true?

    No. In a very small number of AD patients, it is an inherited condition and tends to come on at an earlier age. In motor neurone disease, about 10 % of patients have inherited it,again often with an early onset of the disorder. A few patients with MND have dementia, but this is quite different from AD.

  • I understand the effects of severe electric shock may lead to MND like conditions - would it still be degenerative as in disease, or able to be stabilised and managed as the result of an accident, i.e. not fatal?

    The question of the relationship of electric shock to MND is hotly debated - while there have been several anecdotal reports supporting this relationship, it is not accepted by many neurologists as proven. The cases that have been reported have taken the usual steady progression of the disorder of typical MND. On rare occasions, the spinal cord can be damaged in a severe electric shock and in those cases, the condition stabilises, but this can usually be readily differentiated from MND.

  • Is there any medication of great benefit in Motor Neurone Disease?

    There is no medication that is of any great benefit in Motor Neurone Disease. Riluzole has been suggested to be of benefit in patients with the bulbar form of MND but I would be reluctant to support the use of this medication.

  • Is Motor Neuron Disease hereditary? Are you able to have tests to see if you are likely to develop MND? Is there more than one type of MND?

    In about 10% of patients with Motor Neuron Disease (MND), it is inherited. So in 90% it occurs without any known predisposition or cause. About 20% of the inherited cases are caused by a genetic mutation that can be tested - but as this accounts for only 2% of all patients, it is not a recommended test.

    There are different types of MND - e.g. with predominantly upper motorneuron involvement, with predominantly lower motorneuron involvement or with mainly bulbar involvement. Another variant is lower motorneuron involvement with a benign course.

  • Is a review of Motor Neurone Disease available?

    Yes - from the MND Association of NZ, PO Box 2129, Wellington or www.mdanz.org.nz. A review is in Headlines 2003, Volume 62.