The differential diagnosis of chorea in early adulthood includes Huntington’s disease, spinocerebellar ataxia, HDL2 (Huntington’s disease-like), neuroferritinopathy, Wilson’s disease, NA syndromes, VPS13A‐ and XK‐disease/McLeod, aceruloplasminemia, Niemann‐Pick type C disease, drug-induced, autoimmune (anti-NMDAR encephalitis) causes, and Lesch-Nyhan syndrome [11]. Here, JPH3 is linked to cerebellar ataxia.