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.