“…Patients present with adult-onset progressive ataxia, along with a combination of ophthalmoplegia, spasticity, dystonia, muscular atrophy, or other extrapyramidal signs (Coutinho & Andrade, 1978;Barbeau et al, 1984). The most common affected regions on pathology include the cerebellar dentate nucleus, pallidum, substantia nigra, thalamus, subthalamic nuclei, red nuclei, and, to a lesser extent, the cerebellar cortex (Woods & Schaumburg, 1972;Rosenberg et al, 1976;Romanul et al, 1977;Stefanescu et al, 2015;Hernandez-Castillo et al, 2018;Koeppen, 2018;Wang et al, 2020). Degeneration and other pathologic hallmarks, such as protein aggregate formation, occur fairly late in the course of the disease.…”