Neurofibromatosis Mean paternal age was 32.8 years significantly greater than for control fathers

American Journal of Medical Genetics
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Volume 18, Issue 1 , Pages 169 - 176
Published Online: 3 Jun 2005

Copyright © 1984 Wiley-Liss, Inc., A Wiley Company

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The neurofibromatoses are a group of three genetically distinct but related disorders of the nervous system that cause tumors to grow around the nerves. Tumors begin in the cells that make up the myelin sheath, a thin membrane that envelops and protects nerve fibers, and often spread into adjacent areas. The type of tumor that develops depends on its location in the body and the kind of cells involved. The most common tumors are neurofibromas, which develop in the tissue surrounding peripheral nerves. Most tumors are non-cancerous, although occasionally they become cancerous over time.



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The pathophysiology of neurofibromatosis: IX. Paternal age as a factor in the origin of new mutations
Dr. Vincent M. Riccardi *, Christopher E. Dobson II, Ranajit Chakraborty, Catherine Bontke, John M. Opitz
Neurofibromatosis Program, Baylor College of Medicine, and the Center for Demographic and Population Genetics, Graduate School of Biomedical Sciences, University of Texas Health Science Center, Houston, Texas


*Correspondence to Vincent M. Riccardi, NF Program, Baylor College of Medicine, Texas Medical Center, Houston, TX 77030

Keywords
von Recklinghausen disease • neurofibromatosis • mutation • paternal age • maternal age


Abstract
Von Recklinghausen neurofibromatosis is characterized by a relatively large proportion of apparently nonfamilial cases, presumed spontaneous mutations. This paper analyzes the distribution of paternal and maternal ages for 187 patients with von Recklinghausen disease representing the first definite case in their respective families. Mean paternal age was 32.8 years and mean maternal age was 27.4 years, both being significantly greater than for control populations (P equal to or less than.001). The advanced paternal age was not accounted for by the increase in maternal age. The methodology of controlling the general population paternal ages for each patient's birth year is described.



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Received: 17 September 1983; Revised: 21 November 1983
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