AAN 2022 | Huntington’s disease integrated staging system (HD-ISS) for clinical research

Jee Bang, MD, Johns Hopkins University School of Medicine, Baltimore, MD, discusses the Huntington’s disease integrated staging system (HD-ISS), a new evidence-based classification system for staging the disease. Despite recent advances, the current research paradigm for Huntington’s disease (HD) does not consider the full Huntington’s disease course, including pathophysiology apparent prior to clinical motor symptoms and functional decline. Through empirical analysis of a large cohort of HD patients, the HD-ISS accounts for the entire course of the disease from birth (Stage 0; HD genetic mutation present without any detectable pathological changes), incorporating indicators underlying pathophysiology (Stage 1), clinical signs (Stage 2), and functional changes (Stage 3). This interview took place at the American Academy of Neurology 2022 Congress in Seattle, WA.