<h4>Background</h4>Dentatorubral-pallidoluysian atrophy (DRPLA) is a progressive neurodegenerative disorder caused by expanded CAG repeats in the <i>ATN1</i> gene, characterized by cerebellar ataxia, seizures, tremors, and myoclonus. Here, ATN1 is linked to dentatorubral-pallidoluysian atrophy.