Table 1.
Summary of main findings in the hypothalamus in clinical HD
| Pathology | HD stage | References | |
| Atrophy | ↑ | Premanifest and manifest (VBM) | [29, 31, 121] |
| Grey matter volume | ↓ | Premanifest and manifest (VBM) | [30, 31, 122] |
| = | Premanifest and manifest | [34] | |
| White matter volume | ↓ | Premanifest and manifest (DTI) | [122, 123] |
| Fornix demyelination | ↑ | Premanifest and manifest (DTI,VBM) | [124] |
| NTL volume/neurons | ↓ | Cell counts and volume (HD grade 2–4) | [35–38] |
| PVN neurons | ↓ | Cell counts (HD grade 2–4) | [35] |
| Microglial activation | ↑ | Premanifest and manifest (PET) | [32, 33] |
| Functioning of HPA axis | ↑ | Premanifest and manifest | [107, 109, 125, 126] |
| Circadian rhythm dysfunction | ↑ | Premanifest and manifest | [127] |
| AgRP | = | Immunoreactivity (HD grade 1–4) | [128] |
| BDNF | ↓ | mRNA levels (HD grade 1–4) | [52] |
| CART | ↑ | Immunoreactivity (HD grade 2–4) | [35] |
| = | Immunoreactivity, mRNA levels (HD grade 0, HD grade 1–4) | [40, 52, 128, 129] | |
| CREB-1 | ↑ | mRNA (HD grade 1–4) | [52] |
| CRH | = | Immunoreactivity (HD grade 1–4) | [129] |
| ↑ | mRNA (HD grade 1–4) | [129] | |
| Dopamine D2 receptor | ↓ | Premanifest and manifest (PET), mRNA levels (HD grade 1–4) | [32, 52] |
| FOXO3 | ↑ | mRNA (HD grade 1–4) | [52] |
| MCH | = | Immunoreactivity (HD grade 2–4) | [51, 52] |
| ↓ | mRNA levels (HD grade 1–4) | [52] | |
| NPY | = | Immunoreactivity (HD grade 2–4) | [35] |
| ↓ | Immunoreactivity (HD grade 1–4) | [128] | |
| ↑ | mRNA (HD grade 1–4) | [128] | |
| Orexin | = | Immunoreactivity (HD grade 0) | [40] |
| ↓ | Immunoreactivity, mRNA levels (HD grade 1–4) | [35, 51, 52, 130] | |
| Orexin 2 receptor | ↓ | mRNA levels (HD grade 1–4) | [52] |
| Oxytocin | ↓ | Immunoreactivity (HD grade 0) | [40] |
| ↓ | Immunoreactivity (HD grade 2–4) | [35] | |
| = | Immunoreactivity (HD grade 1–4) | [129] | |
| Prodynorphin | ↓ | mRNA levels (HD grade 1–4) | [52] |
| SIRT1 | ↑ | mRNA (HD grade 1–4) | [52] |
| Somatostatin | ↓ | Immunoreactivity (mid-late HD) | [39] |
| TRH | = | mRNA levels (HD grade 1–4) | [52, 129] |
| Tyrosine hydroxylase | ↓ | mRNA levels (HD grade 1–4) | [52] |
| Vasopressin | ↓ | Immunoreactivity (HD grade 0) | [40] |
| ↓ | Immunoreactivity (HD grade 1–4) | [35, 57] | |
| = | Immunoreactivity (HD grade 1–4) | [129] | |
| = | mRNA levels (HD grade 1–4) | [57] | |
| VIP | ↓ | Immunoreactivity (HD grade 1–4) | [57] |
| = | mRNA (HD grade 1–4) | [57] |
AgRP, agouti-related protein; BDNF, brain-derived neurotrophic factor; CART, cocaine and amphetamine regulated transcript; CREB-1, cyclic AMP-responsive element-binding protein 1; CRH, corticotrophin releasing hormone; DTI, diffusion tensor imaging; FOXO3, forkhead box O3; MCH, melanin-concentrating hormone; NPY, neuropeptide Y; PET, positron emission tomography; SIRT1, sirtuin 1; TRH, thyrotropin-releasing hormone; VBM, voxel based morphometry; VIP, vasoactive intestinal peptide.