Neuropsychological performance of a homozygous patient and his heterozygous brother with Huntington’s disease
| Patient IV.6 | Patient IV.4 | |||||
|---|---|---|---|---|---|---|
| Age at onset (y) | 33 | 30 | ||||
| Disease duration (y) | 3 | 6 | 10 | |||
| No of CAG repeats | 42/46 | 17/48 | ||||
| Global efficiency | ||||||
| Mini Mental State | 29/30 | 0 | 29/30 | 0 | 23/30 | + |
| Mattis Dementia Rating Scale | 138/144 | 0 | 137/144 | 0 | 95/144 | ++ |
| Attentional capacities | ||||||
| WMS - Mental Control | 7/9 | 0 | 5/9 | + | 2/9 | ++ |
| Digit Span WAIS-R | 7/20 | + | 5/20 | + | 1/20 | ++ |
| Stroop Test - Words (T score) | 40 | 0 | 38 | + | ND | |
| Stroop Test - Colours (T score) | 44 | 0 | 38 | + | ND | |
| Stroop Test - Interference (T score) | 32 | + | 38 | + | ND | |
| Trail Making Test A | 52 | 0 | 32 | 0 | ND | |
| Trail Making Test B | 74 | 0 | 66 | 0 | ND | |
| Trail Making Test B - A | 22 | 0 | 34 | 0 | ND | |
| Memory efficiency | ||||||
| WMS - Memory Quotient | 106 | 0 | 99 | 0 | 49 | ++ |
| WMS - Logical Memory | 12 | 0 | 13.5 | 0 | 0 | ++ |
| WMS - Paired Associates | 18,5 | 0 | 13 | 0 | 4 | ++ |
| WMS - Visual Retention | 14 | 0 | 15 | 0 | 2 | ++ |
| California Verbal Learning Test | 60 | 0 | 60 | 0 | ND | |
0 Normal performances, + low performances (1 SD), ++ significant deficits (2 SD), ND: not done, WMS: Wechsler Memory Scale, WAIS-R: Wechsler Adult Intelligence Scale-Revised.