| Striatal degeneration | Moderate to severe neuronal loss in caudate nucleus and putamen |
| Cortical involvement | Variable loss in frontal and temporal cortices |
| White matter changes | Demyelination and axonal loss |
| Intranuclear inclusions | Polyglutamine-containing aggregates (in polyQ HDLs) |
| Databases | OMIMOrphanetClinicalTrialsPubMed |