Deep-brain Stimulation for Dystonia

Current Indications and Future Orientations

Stéphane Thobois; Teodor Danaila; Gustavo Polo; Emile Simon; Patrick Mertens; Emmanuel Broussolle

Future Neurology. 2014;9(1):77-87.

Conclusion

DBS represents a major therapeutic advance for dystonia. However, dystonia consist of a very heterogeneous group of entities, which do not respond to DBS to the same extent. Recognized 'good' indications are primary generalized and segmental (notably cervical) dystonia, and myoclonus and tardive dystonia. Outcomes are much poorer for secondary dystonia, mainly because of the presence of brain lesions, or the progression of the disease. Therefore, for secondary dystonia, indications of DBS should be discussed carefully and on an individual basis. The classical target of stimulation remains the ventrolateral part of the GPi but other targets, such as the subthalamic nucleus, seem promising. The use of combined targets of stimulation to better improve heterogeneous clinical manifestations could also be interesting in the future but new studies are needed to conclude. A summary of the current indications is presented in Table 5.

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Table 1. Deep-brain stimulation for generalized primary dystonia.
Study (year)	Method	Number/etiology	Target	Follow-up	Outcome (%)	Ref.
Coubes et al. (2000)	Open	7 DYT1+	GPi bilat	12 months	BFM: -93.3	[3]
Krause et al. (2004)	Open	10 (4 DYT1+ and 6 DYT1-)	GPi bilat	36 months	BFM: -55.6 (DYT1) BFM: -35.1 (DYT1-)	[4]
Zorzi et al. (2005)	Open	12 DYT1 ?	GPi bilat	20 months	BFM sev: -44.9 BFM disab: - 34.1	[5]
Vidailhet et al. (2005)	Controlled	22 (7 DYT1+ and 15 DYT1-)	GPi bilat	12 months	BFM sev: -51 SF 36: improved by 20	[6]
Kupsch et al. (2006)	Controlled	40 (2 DYT1+ and 38 DYT1-)	GPi bilat	6 months	BFM sev: -39.3 BFM disab: -37.5 SF 36 improved by 29.8	[7]
Cif et al. (2010)	Open	26 DYT1+	GPi bilat	10 years	BFM sev: -72 BFM disab: -58	[8]
Diamond et al. (2006)	Open	11 (5 DYT1+ and 6 DYT1-)	GPi bilat	5 months	UDRS: -15.3	[9]
Egidi et al. (2007)	Open	37 (8 DYT1+ and 29 DYT1-)	GPi bilat	3–84 months	BFM sev: -42 BFM disab: -42	[10]
Isaias et al. (2009)	Open	30 (20 DYT1+ and 10 DYT1-)	GPi bilat	3–8 years	BFM sev: -79.6 BFM disab: -69.2	[11]
Starr et al. (2006)	Open	9 (6 DYT1+ and 3 DYT1-)	GPi bilat	8–14 months	BFM sev: -65.7 (DYT1+) BFM sev: -65 (DYT1-)	[12]
Loher et al. (2008)	Open	2 (1 DYT1+ and 1 DYT1-)	GPi bilat	3 years	BFM sev: - 76 to - 57	[13]
Volkmann et al. (2012)	Controlled	40 (6 DYT1+, 7 DYT1- and 27 DYT1 ?)	GPi bilat	5 years	BFM sev: -61.1	[16]
Vidailhet et al (2007).	Controlled	22 (7 DYT1+ and 15 DYT1-)	GPi bilat	3 years	BFM sev: -58 SF 36: improved by 20	[17]
Mueller et al. (2008)	Open	6 DYT1 ?	GPi bilat	2 years	BFM total: -46	[18]

?: Unkown; BFM: Burke Fahn Marsden scale; bilat: Bilateral; disab: Disabilty; GPi: Internal globus pallidus; sev: Severity; SF 36: Short-form 36 questionnaire; UDRS: Unified Dystonia Rating Scale.

Table 2. Deep-brain stimulation for segmental and focal primary dystonia.
Study (year)	Method	Etiology (n)	Target	Follow-up	Outcome (%)	Ref.
Kiss et al. (2007)	Controlled	Cervical dystonia (10)	GPi bilat	12 months	TWSTRS sev: -42.8 TWSTRS disab: -60 TWSTRS pain: -65 SF 36: improved by 20	[27]
Bittar et al. (2005)	Open	Cervical dystonia (6)	GPi bilat	2 years	TWSTRS sev: -59	[28]
Hung et al. (2007)	Open	Cervical dystonia (10)	GPi bilat	32 months	TWSTRS sev: -54.8 TWSTRS disab: -59.1 TWSTRS pain: -50.4	[29]
Cacciola et al. (2010)	Open	Cervical dystonia (10)	GPi bilat	37.6 months	TWSTRS total: -68	[31]
Skogseid et al. (2012)	Controlled	Cervical dystonia (8)	GPi bilat	30 months	TWSTRS sev: -70 TWSTRS disab: -91 TWSTRS pain: -92	[32]
Walsh et al. (2013)	Controlled	Cervical dystonia (10)	GPi bilat	7.7 years	TWSTRS sev: -51.4 TWSTRS disab: -64.5 TWSTRS pain: -47.7	[33]
Foote et al. (2005)	Open	Meige (1)	GPi bilat	1 year	UDRS: -69	[35]
Houser et al. (2005)	Open	Meige (1)	GPi bilat	6 months	UDRS: -85 BFM: -75	[36]
Ostrem et al. (2007)	Open	Meige (6)	GPi bilat	6 months	BFM: -72 TWSTRS: -54	[37]
Lyons et al. (2010)	Open	Meige (3)	GPi bilat	12 months	BFM sev: -82	[38]
Romito et al. (2010)	Open	Meige (1)	GPi bilat	38 months	BFM sev: -93 TWSTRS: -93	[39]
Sako et al. (2011)	Open	Meige (5)	GPi bilat	49 months	BFM sev: -84 BFM disab: -89	[40]
Groen et al. (2010)	Open	DYT6 (4)	GPi bilat	6 months	BFM sev: -33	[43]
Panov et al. (2012)	Open	DYT6 (3)	GPi bilat	2 years	BFM?	[44]
Goto et al. (2008)	Open	Writer's cramp (1)	Thalamic GPi unilat	3 years	No scale Similar benefit	[63]
Ostrem et al. (2011)	Controlled	Cervical dystonia (9)	STN	12 months	SF 36: improved TWSTRS: -62.9	[67]

BFM: Burke Fahn Marsden scale; bilat: Bilateral; disab: Disability; GPi: Internal globus pallidus; sev: Severity; SF 36: Short-form 36 questionnaire; STN: Subthalamic nucleus; TWSTRS: Toronto Western Spasmodic Torticollis Rating Scale; unilat: Unilateral; UDRS: Unified Dystonia Rating Scale;.

Table 3. Deep-brain stimulation for myoclonus and tardive dystonia.
Study (year)	Method	Etiology (n)	Target	Follow-up	Outcome (%)	Ref.
Cif et al. (2004)	Open	Myoclonus dystonia (1)	GPi bilat	20 months	BFM sev: -84 BFM disab: -89 UMRS: -81	[45]
Azoulay-Zyss et al. (2011)	Open	Myoclonus dystonia (5)	GPi bilat	6–9 months	BFM sev: -85 BFM disab: -66.6 UMRS: -83	[46]
Trottenberg et al. (2001)	Open	Myoclonus dystonia (1)	ViM bilat	2 years	Myoclonus: -80	[64]
Kuncel et al. (2009)	Open	Myoclonus dystonia (1)	ViM bilat	9 months	UMRS: -53	[65]
Damier et al. (2007)	Controlled	Tardive dystonia (10)	GPi bilat	6 months	ESRS: -61 AIMS: -56	[48]
Trottenberg et al. (2005)	Open	Tardive dystonia (5)	GPi bilat	6 months	BFM sev: -87 BFM disab: -96	[49]
Gruber et al. (2009)	Open	Tardive dystonia (9)	GPi bilat	41 months	BFM sev: -83 BFM disab: -67 AIMS: -78.7	[51]

AIMS: Abnormal Involuntary Movement Scale; BFM: Burke Fahn Marsden scale; bilat: Bilateral; disab: Disability; GPi: Internal globus pallidus; sev: Severity; UMRS: Unified Myoclonus Rating Scale; ViM: Ventral intermediate nucleus.

Table 4. Deep-brain stimulation for other secondary dystonia.
Study (year)	Method	Etiology (n)	Target	Follow-up	Outcome (%)	Ref.
Vidailhet et al. (2009)	Controlled	Cerebral palsy (13)	GPi bilat	1 year	BFM sev: -24.4	[52]
Castelnau et al. (2005)	Open	PKAN2 (6)	GPi bilat	6–42 months	BFM sev: -74.6 BFM disab: -53	[53]
Krause et al. (2006)	Open	PKAN2 (1)	GPi bilat	24 months	BFM sev: -23	[54]
Timmermann et al. (2010)	Open	PKAN2 (23)	GPi bilat	9–15 months	BFM sev: -25	[55]
Cif et al. (2007)	Open	Lesch-Nyhan (1)	GPi bilat	28 months	BFM sev: -41	[56]
Havrankova et al. (2009)	Open	Dystonia-deafness (1)	GPi bilat	10 months	BFM sev: -75	[57]
Evidente et al. (2007)	Open	Lubag (1) X-linked dystonia Parkinsonism	GPi bilat	12 months	BFM sev: -76.3	[58]
Martinez-Torres et al. (2009)	Open	Lubag (1) X-linked dystonia Parkinsonism	GPi bilat	12 months	BFM sev: -80	[59]
Wadia et al. (2010)	Open	Lubag (1) X-linked dystonia Parkinsonism	GPi bilat	12 months	BFM sev: -80.4 BFM disab: 66.7	[60]
Roze et al. (2006)	Open	Gangliosidosis GM1 (1)	GPi bilat	12 months	BFM sev: -20	[61]
Sidiropoulos et al. (2013)	Open	Wilson (1)	GPi bilat	20 weeks	BFM sev: -14	[62]

BFM: Burke Fahn Marsden scale; bilat: Bilateral; disab: Disability; GPi: Internal globus pallidus; sev: Severity.

Table 5. Summary of the efficacy of deep-brain stimulation in dystonia according to the etiology.
Type of dystonia	Target of stimulation	Outcome
Primary generalized	GPi	Substantial improvement/long-term benefit/cognitively safe
Cervical	GPi STN	Substantial improvement/long-term benefit Clear benefit
Other focal and segmental dystonia	GPi Vo thalamic nucleus	Positive for Meige syndrome/fewer data for other forms Some positive outcome/much fewer available data
Myoclonus dystonia	GPi Thalamus (ViM) GPi plus ViM	Excellent improvement of both the dystonia and myoclonus Myoclonus improvment but not dystonia GPi DBS > ViM DBS but synergistic effect
Tardive dystonia	GPi Thalamus (ViM)	Excellent improvement of the dystonia and other movement disorders No efficacy
Secondary dystonia	GPi GPi plus STN (gangliosidosis GM1) Limbic plus motor GPi (Lesch–Nyhan) Cortical motor or premotor	Less consistent data/variable outcome/poorer outcome than for primary dystonia Need for larger and controlled studies STN > GPi for this bradykinetic form of dystonia Improvement of the motor plus behavioral component of the disease No consistent result

DBS: Deep-brain stimulation; GPi: Internal globus pallidus; STN: Subthalamic nucleus; ViM: Ventral intermediate nucleus.

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Deep-brain Stimulation for Dystonia

Current Indications and Future Orientations

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