Parkinsonism in Spinocerebellar Ataxia

Table 1

Literature review: clinical and genetic characteristics of SCA2, SCA3, and SCA17 with parkinsonism.

Study	Country	Parkinsonism type	Prevalence, %	Number of study population	Number of affected patients	Onset age, y	Expansion number of patients	Levodopa response	Cerebellar signs	Noticeable clinical features	Long term follow-up complications
SCA2
Shan et al. (2001) [4]	Taiwan	Familial	8.69	23 patients in 19 families	2	50/50	36/37	+	−	Both tremor dominant with slow saccade and CIT-PET (+), nystagmus (+) in 1 patient
Kock et al. (2002) [85]	Mixed	Familial DRP	0	64 young onsets, 32 late onsets (age of onset >50)	0
Payami et al. (2003) [6]	USA	Familial DRP	1.5	136	2	36 (Caucasian)/60 (Hispanic)	33/35	+	−	Evidences of peripheral neuropathy (+) in 1 patient	() D+, MF+
Svetel et al. (2003) [86]	Serbia	Familial; YO-DRP	0	40	0
Lu et al. (2004) [10]	Taiwan	Familial	5.38	130 patients in 41 families	7	45.8 ± 13.9	35–38	+	+^a	1 patient with mild slow saccades and 1 patient with combined mild dystonia	D+^c
Simon-Sanchez et al. (2005) [13]	USA	Familial	0.88	114	1	55 (Caucasian)	37	+
Lim et al. (2006) [26]	Singapore	Familial IPD	0	46	0
Charles et al. (2007) [23]	France	Familial ADP	2	178 patients in 164 families	3	29–64 (50.1 ± 13.2)^d	37–39^d	+	−		D 22%,; MF 14%^d
Lin et al. (2007) [60]	Taiwan	Familial	0	13	0
Modoni et al. (2007) [24]	Italy	Familial	2.5	79	1	48	38	+	−	Tremor dominant and CIT-PET (+)	D−, MF−
Wang et al. (2009) [27]	China	Familial PD	1.5	66	2	42/35	36/36	+	−	CIT-PET (+)
Kock et al. (2002) [85]	Mixed	Sporadic DRP	0	174	0
Svetel et al. (2003) [86]	Serbia	Sporadic; YO-DRP	0	45	0
Shan et al. (2004) [11]	Taiwan	Sporadic PD and atypical parkinsonism	0.4	242	1 in PD group and; 0 in atypical group	56	37	+	−	CIT-PET (+)	D+, MF+
Lim et al. (2006) [26]	Singapore	Sporadic; YO-IPD	2.2	45	1	50	36	+	−	Postural instability
Tan et al. (2007) [61]	Singapore	Sporadic atypical parkinsonism patients with poor levodopa response	0	100	0
Lin et al. (2007) [60]	Taiwan	Sporadic	0	60	0
Modoni et al. (2007) [24]	Italy	Sporadic	0	145	0
Kim et al. (2007) [28]	Korea	Sporadic PD	0.43	468	2	70/55	35/34	+	−	Saccadic movement dysfunction, hyporeflexia in 1 patient
Kim et al. (2007) [28]	Korea	Sporadic MSA-P	0.74	135	1	59	32	Minimal	+	MSA-P type; MRI: cerebellar atrophy
Wang et al. (2009) [27]	China	Sporadic	0.5	386	2	29/37	37/36	+	−
Yun et al. (2011) [29]	Korea	Sporadic PD	0	386	0
Yun et al. (2011) [29]	Korea	Sporadic MSA	0.72	138	1	55	32	−	+	MSA-P type; CIT-PET (+); MRI: cerebellar atrophy
SCA3
Svetel et al. (2003) [86]	Serbia	Familial; YO-DRP	0	40	0
Simon-Sanchez et al. (2005) [13]	USA	Familial	0	114	0
Lim et al. (2006) [26]	Singapore	Familial PD	0	46	0
Lin et al. (2007) [60]	Taiwan	Familial	0	13	0
Charles et al. (2007) [23]	France	Familial ADP	0	178 patients in 164 families	0
Wang et al. (2009) [27]	China	Familial	3	66	4	46/25/40/39	65/69/73/67	+	+^b	CIT-PET (+)
Svetel et al. (2003) [86]	Serbia	Sporadic; YO-DRP	0	45	0
Lim et al. (2006) [26]	Singapore	Sporadic; YO-IPD	0	45	0
Lin et al. (2007) [60]	Taiwan	Sporadic	0	60	0
Tan et al. (2007) [61]	Singapore	Sporadic atypical parkinsonism patients with poor levodopa response	0	100	0
Wang et al. (2009) [27]	China	Sporadic	0.8	386	3	67/36/38	58/64/67	+	−
Yun et al. (2011) [29]	Korea	Sporadic PD	0	386	0
Yun et al. (2011) [29]	Korea	Sporadic MSA	0	138	0
SCA17
Hernandez et al. (2003) [43]	USA	Familial	0	51	0
Lim et al. (2006) [26]	Singapore	Familial PD	0	46	0
Charles et al. (2007) [23]	France	Familial ADP	0	178 patients in 164 families	0
Kim et al. (2009) [21]	Korea	Familial PD	7.41	27	2	44/50	43/45	+	−		D+, MF+
Hernandez et al. (2003) [43]	USA	Familial	0	59	0
Wu et al. (2004) [19]	Taiwan	Sporadic PD	0.38	264	1	75	46	+	−
Lim et al. (2006) [26]	Singapore	Sporadic; YO-IPD	0	45	0
Kim et al. (2009) [21]	Korea	Sporadic PD	0.66	904	6	Mean 53.75 (44–61)	43/44	+	−
Kim et al. (2009) [21]	Korea	Sporadic MSA	0.89	223	2	61/54	29–46	−	+
Yun et al. (2011) [29]	Korea	Sporadic PD	0.78	386	3	47/66/62	45/44/43	+
Yun et al. (2011) [29]	Korea	Sporadic MSA	2.89	138	4	54/59/74/62	46/44/43/43	Partial	+	MSA-P type

Mild dysarthria, ataxic gait, and postural instability in the late stage.
^bPositive in 2 patients.
^cPositive in 1 patient.
^dPrevalence in total 9 including affected family members.
DRP: dopa-responsive parkinsonism, YO: young onset, IPD: idiopathic Parkinson disease, ADP: autosomal dominant parkinsonism, MSA-P: parkinsonian variant of multiple system atrophy, D: dyskinesia, and MF: motor fluctuation.
Each patient is separated with slash mark in onset age and expansion numbers.