Sex/age at examination (yr) | F/34 | F/28 | M/25 | M/35 | M/30 |
Age at onset (yr) | | | | | |
Cognitive decline | 9 | 15 | 9 | 12 | 10 |
Gait disturbance | 17 | 18 | 13 | 16 | 15 |
Neuropsychological tests | | | | | |
WAIS-III | | | | | |
Full-scale IQ | 47a | 47a | 50b | 49a | 49a |
Verbal IQ | 45a | 47a | 48b | 44a | 45a |
Performance IQ | 46a | 44a | 51b | 52b | 49a |
MMSE | | | | | |
Full score | 13a | 19b | 19b | N | 14a |
Orientation | 3/10 | 6/10 | 9/10 | | 4/10 |
Attention and calculation | 0/5 | 0/5 | 1/5 | | 0/5 |
Registration | 3/3 | 3/3 | 2/3 | | 2/3 |
Memory (3-object recall) | 2/3 | 2/3 | 1/3 | | 0/3 |
Language and construction | 5/9 | 8/9 | 6/9 | | 8/9 |
Lower limb pyramidal signs | | | | | |
Hyperreflexiac | +++ | +++ | ++++ | ++++ | +++ |
Spasticityd | 2 | 2 | 3 | 4 | 3 |