Suggestive evidence for linkage to chromosome 13qter for autosomal dominant type 1 porencephaly.

Aguglia, U; Gambardella, A; Breedveld, G J; Oliveri, R L; Le Piane, E; Messina, D; Quattrone, A; Heutink, P · Neurology · 2004

case_series · Level IV

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Abstract

A large three-generation family with autosomal dominant type 1 porencephaly from southern Italy was studied. A high rate of miscarriages was observed. Of the nine affected individuals, four displayed a severe phenotype, and five had slight pyramidal signs or mild cognitive abnormalities. The MRI study disclosed unilateral porencephalic cyst, or colpocephaly. A genome-wide screen resulted in suggestive evidence for linkage to chromosome 13qter with a maximum logarithm-of-the-odds score of 3.16, from multipoint analysis, with marker D13S285.

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