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Table 1: Personal profiles and cognitive functions for patients with ARHSP-TCC with SPG11 mutations
Family 1 Family 2
P1 P2 P3 P4 P5
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 47^{^a} 47^{^a} 50^{^b} 49^{^a} 49^{^a}
    Verbal IQ 45^{^a} 47^{^a} 48^{^b} 44^{^a} 45^{^a}
    Performance IQ 46^{^a} 44^{^a} 51^{^b} 52^{^b} 49^{^a}
MMSE 
    Full score 13^{^a} 19^{^b} 19^{^b} N 14^{^a}
    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 
    Hyperreflexia^{^c} +++ +++ ++++ ++++ +++
    Spasticity^{^d} 2 2 3 4 3
Note:—P indicates patient; F, female; M, male; N, not performed; +++, XXX; ++++, XXX; ++, XXX; WAIS-III, Weichsler Adult Intelligence Scale-III; MMSE, Mini-Mental State Examination.
↵a Last 0.1 Percentile.
↵b Last percentile.
↵c Tendon Reflex Grading Scale.
↵d Modified Ashworth Scale.