TABLE 1.
Overview of studies on bilateral pallidotomy for dystonia
| Author | Gender (M/F) | Age at start of disease | Age at surgery | Symptoms pre‐op | Diagnosis | Dystonia type | BFMDRS movement | BFMDRS disability | Staged/simultaneous | Other scores | Score | Follow‐up | Child/adult | Complications | Part of case report/case series | Aftereffects | Relapse/no improvement |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
|
Levi et al (2019) Italy |
F | 2 y | 10 y | Status dystonicus | PKAN classic | Inherited | – | – | Simultaneous | BAD | SD: 32, postop: 32, last: NA | – | Child | – | Series | Ineffective, death due to SD after 35 days | Death |
| F | 4 y | 7 y | Status dystonicus | Post‐infective SD | Acquired | – | – | Simultaneous | BAD | SD: 32, post‐op: 29, last: 31 | 12 mo | Child | – | Series | 1 day | SD resolution | |
| M | 1 y | 9 y | Status dystonicus | – | – | – | – | Staged | BAD | SD: 30, post‐op: 14, last: 15 | 12 mo | Child | – | Series | 35 days | SD resolution | |
| M | 2 y | 8 y | Status dystonicus | PKAN atypical | Inherited | – | – | Simultaneous | BAD | SD: 31, post‐op: 30, last: 30 | 12 mo | Child | – | Series | 15 days | SD resolution | |
| F | 1 y | 19 y | Status dystonicus | Cerebral palsy | Acquired | – | – | Simultaneous | BAD | SD: 31, post‐op: 24, last: 28 | 38 mo | Adult | – | Series | 50 days | Death due to complications unrelated to baseline dystonia | |
| F | 1 y | 10 y | Status dystonicus | GNAO1 | Inherited | – | – | Simultaneous, after failed DBS | BAD | SD: 32, post‐op: 28, last: 28 | 180 mo | Child | – | Series | 60 days | SD resolution | |
| M | 2 y | 16 y | Status dystonicus | Cerebral palsy | Acquired | – | – | Simultaneous | BAD | SD: 30, post‐op: 28, last: 28 | 30 mo | Child | – | Series | 8 days | Death due to complications unrelated to baseline dystonia | |
|
Garone et al (2019) Italy |
M = 7, F = 1, unknown = 1 | Status dystonicus | – | Both acquired and inherited | – | – | – | DSAP | DSAP4 = 1, DSAP5 = 6, unknown = 2 | – | Child | Series | – | SD resolution in all but 1 patient; dystonia relapse in 7 of 9 patients | |||
|
Franzini et al (2018) a Italy |
F | 4 y | 6 y | Status dystonicus | Tuberculous meningoencephalitis | Acquired | Pre‐op: 110, 3 mo: 50 | Pre‐op: 30, 3 mo: 30 | Simultaneous | – | – | 3 mo | Child | – | Report | Second day after surgery: dramatic reduction of dystonic movements | Unknown |
|
Horisawa et al (2018) a Japan |
M | 36 y | 38 y | Camptocormia | Tardive dystonic camptocormia | Acquired | Pre‐op: 3, post‐op: 0 | Pre‐op: 3, post‐op: 0 | Staged | – | – | 18 mo | Adult | – | Report | Complete resolution of symptoms after unknown time parameter | Unknown |
|
Horisawa et al (2018) a Japan |
M | 43 y | 47 y | Embouchure dystonia | Embouchure dystonia | Acquired | – | – | Staged: 6‐mo interval | – | – | 12 mo | Adult | – | Report | Complete resolution of symptoms after unknown time parameter | Unknown |
|
Kohara et al (2017) Japan |
M | – | 32 y | Trunk and upper extremity dystonia | Tardive dystonia | Acquired | Pre‐op: 28.5, post‐op: 1.5, 9 mo: 0 | Simultaneous | – | – | 9 mo | Adult | – | Report | Immediately post‐surgery | Unknown | |
|
Franzini et al (2017) a Italy |
M | – | 9 y | Status dystonicus | – | Idiopathic | – | – | Staged, 2‐week interval | UDRS | Pre‐op: 110, post‐op: 41 | 6 mo | Child | – | Report | Gradual improvement over 2 mo | Unknown |
|
Minkin et al (2017) a Bulgaria |
M | 45 y | 68 y | Focal dystonia | Meige syndrome | Idiopathic | Pre‐op: 26, 6 mo: 3, 24 mo: 3 | – | Staged: 6‐mo interval | – | – | 24 mo | Adult | – | Report | Immediately | Unknown |
|
Horisawa et al (2016) a Japan |
M | 27 y | 36 y | Cervical dystonia | – | Idiopathic | – | – | Simultaneous | TWSTRS and Tsui score | Pre‐op: 12 and 6, 1 week: 1 (both) | 12 mo | Adult | Transient aggressive behavior | Report | Day after surgery | Unknown |
|
Franzini et al (2015) a Italy |
F | Birth | 15 y | Generalized dystonia | Hypoxic event | Idiopathic | Pre‐op: 56, 12 mo: 28 | – | Staged interval unspecified | – | – | 12 mo | Child | – | Report | Few weeks after lesions | Unknown |
|
Marras et al (2014) a Italy |
M | 3 y | 15 y | Status dystonicus | Chromosomopathy | Inherited | Pre‐op: 101, 22 mo: 16 | Pre‐op: 30, post‐op: 30 | Simultaneous | – | – | 22 mo | Child | – | Series | 40 days | Unknown |
| M | 1 mo | 19 y | Status dystonicus | Epileptic encephalopathy | Pre‐op: 84, 21 mo: 4 | Pre‐op: 30, post‐op: 30 | Simultaneous | – | – | 21 mo | Adult | – | Series | 30 days | Unknown | ||
| M | 4 mo | 6.5 y | Status dystonicus | Bilateral striatal necrosis | Inherited | Pre‐op: 60, 15 mo: 57 | Pre‐op: 30, post‐op: 30 | Simultaneous | – | – | 15 mo | Child | – | Series | 21 days | Unknown | |
| M | 8 mo | 12 y | Status dystonicus | Hypoxic event | Acquired | Pre‐op: 77, 15 mo: 44 | Pre‐op: 26, latest follow‐up: 28 | Simultaneous | – | – | 15 mo | Child | – | Series | 60 days | Unknown | |
|
Fonoff et al (2012)a Brazil |
F | 2 y | 23 y | Generalized dystonia | – | Idiopathic | – | – | – | – | – | 24 mo | Unknown | Severe hypophonia | Series | – | Relapse after 2 y |
| M | 11 y | 41 y | Generalized dystonia | – | Idiopathic | – | – | – | – | – | 24 mo | Unknown | – | Series | – | Relapse | |
| F | 40 y | 58 y | Generalized dystonia | – | Acquired | – | – | – | – | – | 24 mo | Unknown | Speech impairment | Series | – | Relapse after 2.5 y | |
| M | 10 y | 20 y | Generalized dystonia | – | Idiopathic | – | – | – | – | – | 24 mo | Unknown | – | Series | – | Relapse after 4.5 y | |
|
Zirn et al (2011) a Germany |
F | 7 y | 15 y | – | TOR1A gene mutation | Inherited | – | – | – | – | – | – | Child | Mutism, dysarthria, dysphagia, hyperhidrosis | Letter to the editor | Immediately, cessation of hyperkinesia | Needs assistance in all aspects of life, however, may be due to disease progression |
|
Hashimoto et al (2010) a Japan |
M | – | 56 y | Tardive jaw‐opening dystonia | Tardive dystonia | Acquired | Motor speech and eating pre‐op: 8, post‐op: 1 | Pre‐op: 8, post‐op: 2 | Simultaneous | – | 24 mo | Adult | – | Report | Immediately | Unknown | |
|
Elkay et al (2009) a United States |
F | – | 21 y | Status dystonicus | Batten disease | Inherited | Pre‐op: 120, 5 mo: 65 | – | Simultaneous | – | – | 6 y | Adult | – | Report | 10 days | Slight opisthotonus but never to prepallidotomy level |
| F | – | 19 y | Generalized dystonia | Batten disease | Inherited | – | – | – | – | – | 6 y | Child | – | Report | Immediately | Relapse after 3 weeks | |
|
Cersosimo et al (2007) a Argentina |
F | 6 y | 19 y | Generalized dystonia | TOR1A gene mutation | Inherited | Pre‐op: 108, 3 mo: 70, 1 y: 42, 8 y: 21 | Pre‐op: 26, 3 mo: 15, 1 y: 9, 8 y: 8 | Simultaneous | – | – | 96 mo | Adult | – | Series | – | Unknown |
| M | 10 y | 14 y | Focal dystonia | TOR1A gene mutation | Inherited | Pre‐op: 42, 3 mo: 10, 5 y: 11, 6 y: 32, 8 y: 50 | Pre‐op: 12, 3 mo: 8, 5 y: 8, 6 y 12, 8 y: 15 | Simultaneous | – | – | 96 mo | Child | Anarthria | Series | – | Relapse after 60 mo | |
|
Teive et al (2005)a Brazil |
M | – | 8 y | Status dystonicus | Cerebral palsy | Acquired | – | – | – | – | – | Child | – | Series | – | Unknown | |
|
Hwang et al (2005) a United States |
M | 6 mo | 6 y | Generalized dystonia | Glutaric aciduria type 1 | Inherited | Pre‐op: 115, 6 mo 98 | – | Staged, 3‐mo interval | Global Dystonia Rating Score | Pre‐op: 98, post op: 78 | 6 mo | Child | – | Report | Immediately | Unknown |
|
Rakocevic et al (2004) a United States |
M | 10 mo | 18 mo | Generalized dystonia | Glutaric aciduria type 1 | Inherited | – | – | Staged | – | – | 24 mo | Child | Left horizontal gaze preference | Report | – | Unknown |
|
Kyriagis et al (2004) a United States |
M | 16 mo | 9 y | Status dystonicus | Hallervorden‐Spatz disease | Inherited | – | – | Simultaneous | – | – | 12 mo | Child | – | Report | 6 mo, with intrathecal baclofen | Alleviation of spasms with bilateral pallidotomy and baclofen |
|
Hutchison et al (2003) a Canada |
– | 4 y | 13 y | Generalized dystonia | TOR1A gene mutation | Inherited | Pre‐op: 70.5, post‐op: 48 | – | – | – | – | – | Child | – | Series | – | Unknown |
| – | 7 y | 14 y | Generalized dystonia | TOR1A gene mutation | Inherited | Pre‐op: 53.5, post‐op: 13.5 | – | – | – | – | – | Child | – | Series | – | Unknown | |
| – | 7 y | 9 y | Generalized dystonia | TOR1A gene mutation | Inherited | Pre‐op: 75, post‐op: 52 | – | – | – | – | – | Child | – | Series | – | Unknown | |
| – | 9 y | 9 y | Generalized dystonia | – | – | Pre–op: 113.5, post–op: not available | – | – | – | – | – | Child | – | Series | – | Unknown | |
| – | 5 y | 16 y | Generalized dystonia | – | Idiopathic | Pre‐op: 49, post‐op: 45, 5 mo: 56 | – | – | – | – | – | Child | – | Series | – | Relapse at 5 mo | |
| Eltahawy et al (2004) a | M | 7 y | 14 y | Generalized dystonia | TOR1A gene mutation | Inherited | Pre‐op: 75, 6 mo: 25 | – | Simultaneous | Global Outcome Score | 6 mo: 4 | 6 mo | Child | Hypophonia | Series | – | Unknown |
| M | 8 y | 15 y | Generalized dystonia | TOR1A gene mutation | Inherited | Pre‐op: 71, 6 mo: 38 | – | Simultaneous | Global Outcome Score | 6 mo: 3 | 6 mo | Child | Dysphonia | Series | – | Unknown | |
| F | 7 y | 17 y | Generalized dystonia | TOR1A gene mutation | Inherited | Pre‐op: 53, 6 mo: 13 | – | Simultaneous | Global Outcome Score | 6 mo: 4 | 6 mo | Child | – | Series | – | Unknown | |
| M | 5 y | 19 y | Generalized dystonia | Idiopathic | Idiopathic | Pre‐op: 49, 6 mo: 40 | – | Simultaneous | Global Outcome Score | 6 mo: 1 | 6 mo | Adult | – | Series | – | Unknown | |
| M | 1 y | 12 y | Generalized dystonia | Glutaric aciduria | Inherited | Pre‐op: 113, 6 mo: 99 | – | Simultaneous | Global Outcome Score | 6 mo: 1 | 6 mo | Child | – | Series | – | Unknown | |
|
Sanghera et al (2003) a United States |
– | 7 y | 15 y | Generalized dystonia | TOR1A gene mutation | Inherited | Pre‐op: 50, post‐op: 12 | – | Simultaneous | UDRS | Pre‐op: 68, post‐op: 12 | 12 mo | Child | – | Series | – | Unknown |
| – | 8 y | 10 y | Generalized dystonia | TOR1A gene mutation | Inherited | Pre‐op: 26, post‐op: 19 | – | Staged | UDRS | Pre‐op: 36, post‐op: 21 | 12 mo | Child | – | Series | – | Unknown | |
| – | 9 y | 51 y | Generalized dystonia | Pre‐op: 57, post‐op: 15 | – | Staged | UDRS | Pre‐op: 83, post‐op: 26 | 12 mo | Adult | – | Series | – | Unknown | |||
| – | 8 y | 13 y | Generalized dystonia | TOR1A gene mutation | Inherited | Pre‐op: 48, post‐op: 17 | – | Simultaneous | UDRS | Pre‐op: 81, post‐op: 20 | 12 mo | Child | – | Series | – | Unknown | |
| – | 12 y | 16 y | Generalized dystonia | TOR1A gene mutation | Inherited | Pre‐op: 27, post‐op: 10 | – | Simultaneous | UDRS | Pre‐op: 41, post‐op: 2 | 12 mo | Child | – | Series | – | Unknown | |
| – | 7 y | 17 y | Generalized dystonia | – | – | Pre‐op: 59, post‐op: 17 | – | Simultaneous | UDRS | Pre‐op: 101, post‐op: 27 | 12 mo | Child | – | Series | – | Unknown | |
| – | 6 y | 15 y | Generalized dystonia | – | – | Pre‐op: 56, post‐op: 45 | – | Simultaneous | UDRS | Pre‐op: 82, post‐op: 74 | 12 mo | Child | – | Series | – | Unknown | |
| – | 9 y | 19 y | Generalized dystonia | – | – | Pre‐op: 56, post‐op: 43 | – | Simultaneous | UDRS | Pre‐op: 86, post‐op: 63 | 12 mo | Adult | – | Series | – | Unknown | |
| – | 5 y | 15 y | Generalized dystonia | – | – | Pre‐op: 49, post‐op: 46 | – | Simultaneous | UDRS | Pre‐op: 78, post‐op: 66 | 12 mo | Child | – | Series | – | Unknown | |
| – | 2 y | 11 y | Generalized dystonia | – | – | Pre‐op: 58, post‐op: 36 | – | Simultaneous | UDRS | Pre‐op: 88, post‐op: 54 | 12 mo | Child | – | Series | – | Unknown | |
| – | 0.4 y | 8 y | Generalized dystonia | – | – | Pre‐op: 31, post‐op: 25 | – | Staged | UDRS | Pre‐op: 51, post‐op: 34 | 12 mo | Child | – | Series | – | Unknown | |
|
Anca et al (2003) a Israel |
F | 7 y | 15 y | Generalized dystonia | TOR1A gene mutation | Inherited | Pre‐op: 78, 1 y: 42, 2 y: 52 | Pre‐op: 28, 1 y: 22, 2 y: 22 | – | – | – | 24 mo | Child | 1 patient of these aphonia after 1 y | Series | – | Significant motor improvement over first 3–4 mo but progressive worsening after 1 y |
| M | 8 y | 11 y | Generalized dystonia | TOR1A gene mutation | Inherited | Pre‐op: unknown, 1 y: 14, 2 y: 14, 3 y: 23, 4 y: 38 | Pre‐op: unknown, 1 y: 4, 2 y: 4, 3 y: 6, 4 y: 9 | – | – | – | 24 mo | Child | 1 patient of these aphonia after 1 y | Series | – | Relapse after 3 y | |
| M | 9 y | 13 y | Generalized dystonia | TOR1A gene mutation | Inherited | Pre‐op: 68, 1 y: 15, 2 y: 25 | Pre‐op: 26, 1 y: 3.5, 2 y: 9 | – | – | – | 48 mo | Child | 1 patient of these aphonia after 1 y | Series | – | Significant motor improvement over first 3–4 mo but progressive worsening after 1 y | |
|
Teive et al (2001)a Brazil |
M | 37 y | 40 y | Generalized dystonia | Trauma | Acquired | Pre‐op: 51, 3 days: 15, 3 m: 51, 6 mo: 51 | – | Simultaneous | – | – | 6 mo | Adult | Track hemorrhages: motor seizure, hemiparesis | Series | Immediately but relapse 3 mo later | Relapse after 3 mo |
| M | 16 y | 35 y | Generalized dystonia | – | – | Pre‐op: 60, 3 days: 52 (stage1) /46 (stage 2), 3 mo: 18, 6 mo: 18 | – | Staged | – | – | 6 mo | Adult | – | Series | Progressive improvement up to 3 mo | Unknown | |
| – | 5 y | – | – | – | – | Pre‐op: 50, 3 days: 50, 3 mo: 28, 6 mo: 28 | – | Simultaneous | – | – | 6 mo | Child | – | Series | Progressive improvement up to 3 mo | Unknown | |
| M | 23 y | 28 y | Generalized dystonia | – | – | Pre‐op: 48, 3 days: 40, 3 mo: 6, 6 mo: 4 | – | Simultaneous | – | – | 6 mo | Adult | Transient lethargy | Series | Progressive improvement over 6 mo | Unknown | |
| ‐ | 21 y | – | – | – | – | Pre‐op: 28, 3 days: 24, 3 mo: 12, 6 mo: 12 | – | Simultaneous | – | – | 6 mo | Adult | – | Series | Progressive improvement up to 3 mo | Unknown | |
|
Cubo et al (2000) a United States |
F | 4 y | 13 y | Generalized dystonia | Westphal variant of Huntington's disease | Inherited | Pre‐op: 56, 3 mo post‐op: 49 | – | Simultaneous | – | – | 3 mo | Child | Edema on the right capsule: seizures | Report | Little improvement over 3 mo | Disease progression |
|
Iacono et al (1996) a United States |
M | 8 y | 17 y | Generalized dystonia | TOR1A gene mutation | Inherited | – | – | Simultaneous | – | – | 12 mo | Child | – | Report | Immediately | Unknown |
|
Vitek et al (1998) a United States |
– | – | – | Generalized dystonia | – | – | Pre‐op: 34, 1 mo: 10 | Pre‐op: 10, 1 mo: 3 | – | – | – | 2 mo | Unknown | – | Series | – | Unknown |
| – | – | – | Generalized dystonia | – | – | Pre‐op: 31, 2 mo: 5 | Pre‐op: 8, 2 mo: 0 | – | – | – | 2 mo | Unknown | – | Series | – | Unknown | |
| – | – | – | Generalized dystonia | – | – | Pre‐op: 41, 1 week: 6 | Pre‐op: 8.5, 1 week: 2 | – | – | – | 2 mo | Unknown | – | Series | – | Unknown | |
|
Ondo et al (1998) a United states |
M | 10 y | 51 y | Generalized dystonia | – | – | Pre‐op: 57, post‐op: 9 | Pre‐op: 16, post‐op: 8 | Staged | UDRS, ADL | Pre‐op: 83, post‐op: 20, ADL = pre‐op: 16, post‐op: 8 | 6 mo | Adult | – | Series | – | Unknown |
| – | 13 y | 18 y | Generalized dystonia | Trauma | Acquired | Pre‐op: 56, post‐op: 46 | Pre‐op: 18, post‐op: 16 | Simultaneous | UDRS, ADL | Pre‐op: 86, post‐op: 58, ADL = pre‐op: 18, post‐op: 16 | 6 mo | Adult | – | Series | – | Unknown | |
| M | 10 y | 16 y | Generalized dystonia | Hypoxic event | Acquired | Pre‐op: 59, post‐op: 17 | Pre‐op: 19, post‐op: 9 | Simultaneous | UDRS, ADL | Pre‐op: 101, post‐op: 27, ADL = pre‐op: 19, post‐op: 9 | 6 mo | Child | – | Series | 2 days | Unknown | |
| F | 7 y | 14 y | Generalized dystonia | Genetic | Inherited | Pre‐op: 50, post‐op: 17 | Pre‐op: 18, post‐op: 7 | Simultaneous | UDRS, ADL | Pre‐op: 68, post‐op: 21, ADL = pre‐op: 18, post‐op: 7 | 6 mo | Child | Transient lethargy | Series | Gradual improvement over 3 mo | Partial recurrence after 6 mo | |
| F | 8 y | 13 y | Generalized dystonia | TOR1A gene mutation | Inherited | Pre‐op: 48, post‐op: 17 | Pre‐op: 17, post‐op: 6 | Simultaneous | UDRS, ADL | Pre‐op: 81, post‐op: 20, ADL = pre‐op: 17, post‐op: 6 | 6 mo | Child | – | Series | Within 3 weeks | Unknown | |
|
Weetman et al (1997) a United Kingdom |
M | – | 31 y | Generalized dystonia | Tardive dystonia | Acquired | Pre‐op: 76, 8 mo: 21 | Pre‐op: 22, 8 mo: 4 | Simultaneous | Obeso scale | Grade 2 | 8 mo | Adult | – | Report | Immediately | Slight recurrence |
|
Lin et al (1999)a,b Taiwan |
M | – | 29 y | Generalized dystonia | Perinatal asphyxia | Acquired | Pre‐op: 51, 3 mo: 37, 6 mo: 33.5, 1 y: 33.5 | – | – | – | – | 12 mo | Adult | – | Report | Improvement over 6 mo | Unknown |
|
Taiwan |
F | 30 y | 36 y | Generalized dystonia | Dystonia due to hypovolemic shock | Acquired | Pre‐op: 74, 1 mo: 47, 3 mo: 34, 6 mo: 28, 9 mo: 28 | Pre‐op: 20, 1 mo: 12, 3 mo: 10, 6 mo: 9, 9 mo: 9 | – | – | – | 9 mo | Adult | Transient right facial weakness | Report | Improvement over 9 mo | Unknown |
|
Lin et al (2001) b Taiwan |
n = 18, 8 men, 10 women | Average = 24.8 y | BFMDRS movement | Generalized dystonia | – | – | 13% decrease | 9% decrease | 14 simultaneous, 4 staged | – | – | 12 mo | – | Transient adverse effects in 7 of 18: urinary incontinence (2), visual field defects (2), hemiparesis (2), unsteady gait (1), fever (1) | Series | – | Unknown |
| Khandelwal et al (2018) | M | – | 48 y | Cervical dystonia | Cervical dystonia | – | – | – | Simultaneous | – | – | – | Adult | Transient bilateral mydriasis and visual field defects | Report | – | Unknown |
a
Included in meta‐analysis.
b
Overlapping patient.
Abbreviations: BFMDRS, Burke‐Fahn‐Marsden Dystonia Rating Scale; DBS, deep brain stimulation; ADL, activities of daily living; UDRS, Unified Dystonia Rating Scale; BAD, Barry Albright Dystonia; SD, status dystonicus; TWSTRS, Toronto Western Spasmodic Torticollis Rating Scale; PKAN, Pantothenate Kinase‐Associated Neurodegeneration; DSAP, Dystonia Severity Action Plan.