Parental sex effect in familial amyotrophic lateral sclerosis.
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.
Leone
M
M
Divisione di Neurologia, Ospedale Civile, Ivrea, Italy.
D000328:Adult; D000368:Aged; D000375:Aging / Q000502:physiology; D000690:Amyotrophic Lateral Sclerosis / Q000235:genetics* / Q000503:physiopathology; D005220:Fathers; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009035:Mothers