Table 1.

Clinical characteristics of manifesting gene carriers^a

Group	Participation	Age, years	Symptom duration, years	Distribution (body part)	Burke–Fahn– Marsden motor score	Medications	MSEQ/CCW pace (Hz)
DYT1
1	1	23	13	Generalized (Lg, rA)	17	THX, baclofen	1
2	–	24	14	Generalized (C, A, Lg, T)	68	Ethopropazine, baclofen	2
3	1,2,3	28	11	Focal (rA)	1	THX	1
4	3	31	15	Focal (rA)	4	None	1
5	1,2,3	39	31	Generalized (A, Lg, T)	33	THX, baclofen, zonisamide	1
6	1,2,3	41	32	Generalized (C, A, Lg, T)	54	THX	1
7	1,2,3	42	36	Generalized (C, A, Lg, T)	17	THX, levodopa, zonisamide	1
8	1,2	57	50	Generalized (C, rA, rLg)	23	Topiramate	1.16
9	1,2,3	57	45	Generalized (C, rA, Lg)	6.5	Levodopa	1.3
10	1,2,3	64	54	Generalized (C, A, Lg, T)	35	THX	2
11	1,2,3	65	57	Multifocal (A, Lg)	19	None	1
DYT6
1	2,3	19	11	Generalized (C, A, Lg, T)	23	THX, BTX	1
2	2	19	3	Focal (C)	2	None	1
3	2,3	23	11	Generalized (C, rA, Lg)	10.5	THX, BTX	1
4	2,3	45	20	Segmental (A)	5	None	1
5	2,3	45	34	Generalized (C, A, Lg, T)	51	Pregabalin,	1
6	2,3	48	28	Focal (C)	6	THX	1
7	2	49	14	Segmental (A)	6	None	1

a All subjects participated in psychophysical testing. 1 = H₂¹⁵O PET study; 2 = raclopride PET study and 3 = 3T diffusion tensor imaging MRI. A = arm(s); BTX = botulinum; C = craniofacial; l = left; Lg = leg(s); r = right; T = trunk; THX = trihexyphenidyl.