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Anticipation in myotonic dystrophy: a parental-sex-related phenomenon
A López de Munain1, A Blanco, J I Emparanza
1Department of Neurology, Hospital Ntra. Sra de Aránzazu, San Sebastián, Guipúzcoa, Spain.
Neuroepidemiology
|January 1, 1994
Abstract:
We report anticipation in an extensive sample of myotonic dystrophy (MyD) kindreds taken from an epidemiological survey recently conducted in Guipúzcoa, Spain. Analysis of the parent-child pairs ascertained showed a mean anticipation of 2.86 decades (range 0-6). Greater anticipation occurred when the transmissor parent was the mother. These results suggest a possible sex-related effect in the transmission of the MyD gene, and are in agreement with recent discoveries at the molecular level.