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Since a parental sex effect has been reported in Huntington's disease,we looked to see whether a similar effect is apparent in adult(autosomal dominant)familial ALS.We analyzed the data for 145 patients,with an onset age range of 20 to 68 years and a known affected parent(AP),from 52 families described in the literature.There was a significant increase in the percentage of patients inheriting the gene from an affected mother as a function of the age at onset.There was also a significant correlation between AP and offspring age at onset only when the AP was the mother. |
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