VPS13A disease (chorea-acanthocytosis, ChAc, OMIM: 200150; Jung et al., 2011; Peikert et al., 2023) and Huntington’s disease (HD, OMIM: 143100; Ross et al., 2014) are two basal ganglia disorders that are often clinically difficult to distinguish because they have similar symptoms, magnetic resonance imaging findings (Suzuki et al., 2020) and neuropathological features with selective degeneration of the medium spiny neurons (MSNs) in the striatum (Liu et al., 2019). Here, VPS13A is linked to Huntington disease.