Skip to main content
. 2019 Apr 1;76(6). doi: 10.1001/jamaneurol.2019.0423

Table 4. Known Prevalence of Polyglutamine Diseases Compared With the Prevalence Among Carriers of CAG Repeat Lengths Within the Disease-Causing Range.

Disease Disease Prevalence per 100 000a Genotype Frequency per 100 000
SCA1 0.2-2.0 65.8
SCA2 0.1-5.8 59.1
SCA3 0.6-0.8 14.8
SCA6 0.0-0.5 7.3
SCA7 0.1-0.2 0
SCA17 0.2 1052.6
HD 10.6-13.7 139.0
DRPLA 0.5b 0
SBMA 0.3c 16.0c

Abbreviations: CAG, cytosine-adenine-guanine; DRPLA, dentatorubral-pallidoluysion atrophy; HD, Huntington disease; SBMA, spinal bulbar muscular atrophy; SCA, spinocerebellar ataxia.

a

Derived from the literature.3,4,5,6,7,8,9,10,11

b

Prevalence among Japanese.

c

Prevalence among men.