Participant demographic and clinical characteristics across all groups
| Group; mean ± SD (range)* | |||
|---|---|---|---|
| Characteristic | Controls, n = 18 | Pre-HD, n = 25 | Symp-HD, n = 23 |
| Sex, M:F | 4:14 | 9:16 | 13:10 |
| Age, yr | 45.54 ± 13.7 (27–75) | 42.86 ± 9.2 (26–68) | 55.98 ± 9.4 (42–73)**†† |
| Estimated full-scale IQ† | 116.23 ± 10.9 | 116.69 ± 11.3 | 116.62 ± 11.1 |
| UHDRS‡ | — | 1.0 ± 1.2 (0–4) | 26.50 ± 18.2 (7–79)** |
| CAG repeats§ | — | 42.50 ± 1.9 | 42.65 ± 2.0 |
| DBS¶ | — | 287.75 ± 61.8 | 389.6 ± 65.0** |
| Estimated YTO | — | 13.48 ± 7.4 | — |
| Duration of illness, yr | — | — | 5.07 ± 1.5 |
CAG = cytosine-adenine-guanine; DBS = disease burden score; pre-HD = premanifest gene carriers of Huntington disease; SD = standard deviation; symp-HD = symptomatic Huntington disease; UHDRS = Unified Huntington’s Disease Rating Scale; YTO = years to onset.
↵* Unless otherwise indicated.
↵† Estimated full scale IQ + National Adult Reading Test (NART) error score.
↵‡ Pre-HD: UHDRS < 5; symp-HD: UHDRS ≥ 5.
↵§ Number of repeats > 40 is full penetrance.
↵¶ DBS = (CAG – 35.5) × age.
↵** Symp-HD – pre-HD, p < 0.01.
↵†† Symp-HD – controls, p < 0.01.