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NEUROLOGY 2005;64:1628-1631
© 2005 American Academy of Neurology
Brief Communications

Patterns of inheritance in familial ALS

Marcus Bradley, MRCP, Lloyd Bradley, MRCP, Jackie de Belleroche, PhD, FRCPath and Richard W. Orrell, MD, FRCP

From the Department of Clinical Neurosciences (Drs. M. Bradley, L. Bradley, and Orrell), Royal Free and University College Medical School, University College London, London, England; and Department of Neuromuscular Diseases (Dr. de Belleroche), Imperial College School of Medicine, London, England.

Address correspondence and reprint requests to Dr. Richard W. Orrell, Department of Clinical Neurosciences, Royal Free and University College Medical School, University College London, Rowland Hill Street, London NW3 2QG, England; r.orrell{at}rfc.ucl.ac.uk

We investigated 185 families with ALS for evidence of anticipation and mitochondrial inheritance. Although initial analysis demonstrated significant anticipation of age at death between generations in patients with familial ALS, further analysis demonstrated features of regression to the mean, suggesting that the perceived differences are the result of bias. In addition, there was no evidence of an effect of preferential maternal inheritance, which would have supported transmission of mitochondrial DNA mutations.

Supported by the Motor Neurone Disease Association, The Peel Medical Research Trust, and the Peter Samuel Royal Free Fund.

Received November 18, 2004. Accepted in final form January 18, 2005.






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