Table 1

Clinical features in 17 German patients with SCA6 (in order of increasing repeat length)

		Family - patient
		A-1	B-1	C-1	D-1	E-1	F-1	G-1	E-2	H-1	A-2	J-1	K-1	K-2	L-1	L-2	M-1	N-1
CAG	22	22	22	22	22	22	22	22	22	22	22	23	23	26	26	27	28
Age at onset	71	60	60	59	59	58	54	54	53	50	47	57	47	31	30	35	32
Duration	18	23	12	7	8	1	4	2	9	6	3	12	13	27	1	17	17
Age at examination	89	83	72	66	67	59	58	56	62	56	50	69	60	58	31	52	49
Sex	M	F	F	F	M	M	M	F	M	M	F	F	M	F	F	M	M
Cerebellar	+++	+++	+++	++	++	+	+	+	++	+	+	++	++	+++	+	+++	++
Gait ataxia	+++	+++	+++	++	++	+	+	+	++	+	+	++	+	+++	+	+++	+
Upper limb ataxia	++	++	+++	+	+	(+)	(+)	+	+	+	(+)	++	++	++	(+)	++	++
Dysarthria	++	++	++	+	+	0	+	+	+	+	+	++	+	++	+	++	+
Oculomotor disturbance	++	++	++	++	+	(+)	++	+	++	++	++	++	++	++	++	++	++
Swallowing problems	+	++	+	+	0	0	0	0	0	+	0	+	+	0	0	+	0
External ophthalmoplegia	++		++	0	+	0	0	+		0	0	0	0	+	0	0
Extrapyramidal signs	0	0	T	0	0	0	0	0	A,R	0	0	0	0	0	0	0	0
Pyramidal affection	0	0	0	+	0	0	0	+	0	+	0	+	(+)	+	0	+	(+)
Peripheral neuropathy	0	+	+	0	++	0	+	(+)	0	0	0	+	(+)	0	0	0	0
MRI			CA	CA	CA	CA	CA	CA^*	CA	CA^*		CA	CA^*		CA	CA	CA

0=normal; (+)=minimal; +=mild; ++=moderate; +++=severe; A=akinesia; R=rigidity; T=tremor; CA=cerebellar atrophy; ^*=mild cerebral atrophy additionally to CA.