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. 2021 Oct 12;37(1):16–24. doi: 10.1002/mds.28823

TABLE 1.

Neuropathological severity of the striatum in Huntington disease brains 14 , 33

Vonsattel grading system JHD brains (N = 50) AHD brains (N = 1300)
Grade 0 Not reported 1%
Grade 1 10% a 4%
Grade 2 Not reported 16%
Grade 3 26% 53%
Grade 4 64% 28%
a

Ninety percent of grade 1 juvenile‐onset Huntington disease (JHD) brains were from JHD patients who committed suicide and therefore do not reflect end‐stage disease. For the adult‐onset Huntington disease (AHD) brains, these percentages are unknown/not provided.