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When correlation was made for age at death,greater numbers of trinucleotide repeats were associated with greater neuronal loss,in both the caudate(r=0.9641,p,0.001)and the putamen(r=0.9652,p<0.001).When correction was made for disease duration,the correlation was again significant,for both the caudate(r=0.6396,p<0.01)and the putamen(r=0.6710, p<0.001).This suggests that in HD,longer trinucleotide repeat length is associated with a faster rate of deterioration and greater pathologic severity.A comparison of trinucleotide repeat length in different brain regions in 4 of the HD postmortem cases associated with greater numbers of repeats consistently demonstrated fewer repeats in the cerebellum than in the frontal cortex,striatum or blood. |
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