Clinical, genetic, and neuroradiologic information of patients with DRPLA

	Juvenile-Onset Cases				Adult-Onset Cases
	Patient No.:	1	2	3	4	5	6
Age (y)		26	28	29	53	56	53
Sex		F	M	F	M	M	F
CAG repeat		65	65	60	63	60	59
Duration (y)		10	15	10	10	15	10
Onset (y)		16	13	19	43	41	43
Epilepsy		+++	plus;+	+	−	−	−
Dementia		+	+	+	++	++	+++
Ataxia		++	+	−	++	++	++
Chorea		+	−	−	+	++	+
MR images
Atrophy
Cerebrum		−	−	−	+	+	+
Cerebellum		+	+	+	++	+	+
T2 high-signal GP		−	−	−	−	−	−
Thalamus		−	−	−	−	−	−
White matter		−	−	−	+	++	++

Note:—DRPLA indicates dentatorubral-pallidoluysian atrophy; GP, globus pallidus; −, not apparent; +, mild; ++, moderate; +++, severe.