Age at examination (years) | 42 | 39 | 36 | 30 | 22 |
Onset | 6 years | 6 years | 6 years | 6 years | 11 years |
Dysarthria | +++ | +++ | +++ | +++ | − |
Tongue tremors | + | + | + | + | + |
Atrophy of small hand muscles | +++ | + | +++ | +++ | +++ |
Muscle tone | Spastic LL | Spastic LL | Spastic LL | Spastic LL | Slightly spastic LL |
Knee reflex | +++ | +++ | +++ | +++ | − |
Clonus | +++ | + | + | − | − |
Extensor plantar response | +++ | +++ | +++ | − | − |
Gait | Spastic ataxic | Spastic | Spastic | Spastic | Spastic |
Emotional lability | + | +++ | + | +++ | |
IQ | Total 55 | Total 61 | Total 54 | Total 56 | Total 68 |
| Verbal 55 | Verbal 55 | Verbal 52 | Verbal 48 | Verbal 73 |
| Performance 61 | Performance 72 | Performance 73 | Performance 73 | Performance 64 |