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Hereditary Ataxia and the Sixth Chromosome
Arnulf H. Koeppen, MD;
H. Werner Goedde, PhD;
Christian Hiller, MD;
Ludwig Hirth, MD;
Heide-G. Benkmann
Arch Neurol. 1981;38(3):158-164.
Abstract
• Possible linkage of the gene or genes for dominant hereditary ataxia and three genetic markers on the short arm of the sixth chromosome (HLA, properdin factor B [Bf], and glyoxalase I) was investigated in five families. Logarithmic odds (lod scores) were calculated for the linkages and found to be either inconclusive or in favor of nonlinkage. Caution is advised in the summing of lod scores for separate families because of the wide spectrum of clinical and anatomical manifestations of dominant hereditary ataxia. Three families with recessive hereditary ataxia were also studied. Identical haplotypes occurred in affected and unaffected siblings. It did not appear likely that the recessive genes of the parents were transmitted in linkage with the markers on the short arm of the sixth chromosome.
Author Affiliations
From the Institute of Human Genetics (Drs Goedde and Hirth and Ms Benkmann), and the Institute for Transplantation Immunology (Dr Hiller), the University of Hamburg, Germany; and the Department of Neurology, Albany Medical College, and the Neurology Service, Veterans Administration Medical Center, Albany, NY (Dr Koeppen).
Footnotes
Accepted for publication July 7, 1980.
Reprint requests to Neurology Service, Veterans Administration Medical Center, Albany, NY 12208 (Dr Koeppen).
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