Sex Differences in Huntington’s Disease: Considerations for Clinical Care and Research Trials

Sex is probably the most obvious distinguishing characteristic in humans. Accumulating evidence demonstrates significant sex differences in brain anatomy, physiology, and behavior from early neurodevelopmental stages to senescence. However, sex differences have not been well explored by studies describing the natural history of Huntington’s Disease (HD), partly because of the disease’s autosomal dominant nature with equal penetrance and prevalence in both sexes. Data about sex differences in HD is still scarce and thus far based on sex-related disease anticipation and recent studies showing a more severe phenotype in females. In this chapter, we will discuss sex-related differences in the presentation and progression of HD, their impact on biomarkers, and how this emerging knowledge can influence clinical practice and research.