Scottish Motor Neurone Disease Association

Scottish Motor Neurone Disease Association	Additional Information
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The Scottish Motor Neurone Disease Association would like to thank Dr Stuart Neilson and Dr Frank Clifford Rose for permission to use the following extracts from their book. “Motor Neurone Disease” At Your Fingertips Guide”. Class Publishing (London) Ltd 2003 ISBN: 859590470 How long does diagnosis take? Who is at greatest risk of developing MND? What are the symptoms and how fast does MND progress? Are other members of my family likely to get MND too? Are there any drugs for treating MND? What are Stem Cells? Is it safe for me to continue regular exercise? Are other members of my family likely to get MND too? One form of MND, amyotrophic lateral sclerosis, exists both in a form that relatives get (‘familial’) and a random (‘sporadic’) form. The familial form of ALS is associated with symptoms that start early but progress slowly; it often affects several family members over many generations within a single family. ‘Sporadic’ ALS is by far the more common, accounting for 90% of all people with ALS. Although genetic changes may be a cause of MND in these people, it is not clear that any risk of getting MND can be inherited by other members of the family. If neither of your natural parents, nor any other blood relative has MND, then it is highly unlikely that your form of MND can be passed on, but equally, like any other random event, there can be more than one case of MND in a family by chance alone. We estimate that about 4% of all people with MND would be expected, by chance alone, to have a relative with MND, when great-grandparents, aunts, uncles and cousins are included as well as immediate family. General research has been gathering pace over the last decade, confirming there is some genetic involvement in MND. About 10% of cases of ALS have a ‘familial’ origin, usually occurring in clusters within affected families. Outside these affected families, the familial pattern (if any) is difficult to interpret. Advances in genetic analysis have led to the discovery of ‘novel mutations’, in other words changes to genes following conception, but not inherited from either parent. A form of one particular gene (the superoxide dismutase-1 gene)is found in about 20% of familial cases of ALS and in some people with ‘sporadic’ ALS. It seems likely that MND is not associated with a single genetic cause but with a range of genes that independently alter the risk of MND and are required in certain combinations before MND is likely to occur. It is not possible, at present, to give a genetic test to ascertain the likelihood of future MND because the two major genes already known account for only a small proportion of all people with MND. Even amongst people with familial ALS, only 20% have the variant gene (superoxide dismutase-1) associated with MND, so any test results would be, at best, inconclusive. In time it is likely that comprehensive and reliable tests will be developed.
Scottish Motor Neurone Disease Association, 76 Firhill Road,, Glasgow, G20 7BA, Tel: 0141 945 1077, Fax: 0141 945 2578, email: The Scottish Motor Neurone Disease Association is a company limited by guarantee, registered in Scotland, number 217735. It is registered as a Scottish Charity with the Office of the Scottish Charity Regulator (OSCR), SCO 02662.

The Scottish Motor Neurone Disease Association would like to thank Dr Stuart Neilson and Dr Frank Clifford Rose for permission to use the following extracts from their book. “Motor Neurone Disease” At Your Fingertips Guide”. Class Publishing (London) Ltd 2003 ISBN: 859590470

Are other members of my family likely to get MND too?

One form of MND, amyotrophic lateral sclerosis, exists both in a form that relatives get (‘familial’) and a random (‘sporadic’) form. The familial form of ALS is associated with symptoms that start early but progress slowly; it often affects several family members over many generations within a single family. ‘Sporadic’ ALS is by far the more common, accounting for 90% of all people with ALS. Although genetic changes may be a cause of MND in these people, it is not clear that any risk of getting MND can be inherited by other members of the family. If neither of your natural parents, nor any other blood relative has MND, then it is highly unlikely that your form of MND can be passed on, but equally, like any other random event, there can be more than one case of MND in a family by chance alone. We estimate that about 4% of all people with MND would be expected, by chance alone, to have a relative with MND, when great-grandparents, aunts, uncles and cousins are included as well as immediate family.

General research has been gathering pace over the last decade, confirming there is some genetic involvement in MND. About 10% of cases of ALS have a ‘familial’ origin, usually occurring in clusters within affected families. Outside these affected families, the familial pattern (if any) is difficult to interpret. Advances in genetic analysis have led to the discovery of ‘novel mutations’, in other words changes to genes following conception, but not inherited from either parent. A form of one particular gene (the superoxide dismutase-1 gene)is found in about 20% of familial cases of ALS and in some people with ‘sporadic’ ALS. It seems likely that MND is not associated with a single genetic cause but with a range of genes that independently alter the risk of MND and are required in certain combinations before MND is likely to occur.

It is not possible, at present, to give a genetic test to ascertain the likelihood of future MND because the two major genes already known account for only a small proportion of all people with MND. Even amongst people with familial ALS, only 20% have the variant gene (superoxide dismutase-1) associated with MND, so any test results would be, at best, inconclusive. In time it is likely that comprehensive and reliable tests will be developed.