Table 2

Tremor distribution and outcome according to the treatment

Reference	Dystonia type	Dystonia duration (years)	Dystonia distribution	Body site	Tremor site	Total number of cases	Case No	Sex	Age	Treatment (daily dose in mg)	Outcome (or % of improvement when available)
Drugs
27	Idiopathic	?	Focal	Neck	Head	3	?	?	?	Trihexyphenidyl (6–8), levodopa (1500), diazepam (2)	Lower tremor frequency in two cases, no more tremor in one case
30	Idiopathic	7	Focal	Arm	Arm	8	1	M	46	Anticholinergics*	25
	Idiopathic	7	Focal	Arm	Arm		1	M	46	β-blockers*	25
	Idiopathic	1.5	Focal	Arm	Arm		2	M	52	Anticholinergics*	75
	Idiopathic	9	Focal	Arm	Arm		3	M	43	Anticholinergics*	100
	Idiopathic	9	Focal	Arm	Arm		3	M	43	β-blockers*	50
	Idiopathic	12	Focal	Arm	Arm		4	F	55	Anticholinergics*	25
	Idiopathic	12	Focal	Arm	Arm		4	F	55	β-blockers*	50
	Idiopathic	0.5	Focal	Arm	Arm		5	M	36	Anticholinergics*	0
	Idiopathic	5	Focal	Arm	Arm		6	M	18	β-blockers*	100
	Idiopathic	5.5	Focal	Arm	Arm		7	M	55	Anticholinergics*	0
	Idiopathic	3	Focal	Arm	Arm		8	M	21	Anticholinergics*	25
	Idiopathic	3	Focal	Arm	Arm		8	M	21	β-blockers*	100
22	Idiopathic	8	Focal	Neck	Head	2	1	M	26	Carbamazepine, diazepam, propranolol	0
	Idiopathic	8	Focal	Neck	Head		1	M	26	Clonazepam (1)	Marked decrease in frequency and duration of attacks
	Idiopathic	10	Focal	Neck	Head		2	M	30	Acetazolamide, trihexyphenidyl, carbamazepine, clonazepam, diazepam	0
31	Idiopathic	?	Focal	Hand	PWT	4	?	?	?	Propranolol	Improvement
	Idiopathic	?	Focal	Hand	PWT	8	?	?	?	Propranolol	No change
	Idiopathic	?	Focal	Hand	PWT	3	?	?	?	Primidone	Improvement
	Idiopathic	?	Focal	Hand	PWT	1	?	?	?	Primidone	No change
	Idiopathic	?	Focal	Hand	PWT	3	?	?	?	Trihexyphenidyl	Improvement
	Idiopathic	?	Focal	Hand	PWT	1	?	?	?	Tetrabenazine	Improvement
	Idiopathic	?	Focal	Hand	PWT	1	?	?	?	Orphenadrine	Improvement
36	Idiopathic	12	Segmental	Neck, arms, trunks	Head, arms, trunk	3	1	M	22	Clonazepam (0.5)	Significant reduction in tremor
	Idiopathic	0.1	Focal	Neck	Head		2	M	18	Clonazepam (3)	100
	Idiopathic	2	Focal	Arm	Arm		3	M	16	Clonazepam (0.5)	100
32	Idiopathic	18	Multifocal	Arms	Arms	1	1	M	34	Trihexyphenidyl (10)	Clear improvement in symptoms
35	Idiopathic	10.7	Multifocal	Neck, legs	Head	1	1	M	11	Trihexyphenidyl (4)	Slight improvement
	Idiopathic	10.7	Multifocal	Neck, legs	Head		1	M	11	Clonazepam (3)	Slight improvement
	Idiopathic	10.7	Multifocal	Neck, legs	Head		1	M	11	Timolol (15)	Sustained improvement
	Idiopathic	10.7	Multifocal	Neck, legs	Head		1	M	11	Propranolol (40)	0
	Idiopathic	10.7	Multifocal	Neck, legs	Head		1	M	11	Primidone (150)	0
42	Idiopathic	19	Segmental	Eyes, jaw, neck	Head	5	1	M	62	Riluzole (100)	58
	Idiopathic	15	Focal	Neck	Head		2	M	58	Riluzole (100)	50
	Idiopathic	25	Focal	Neck	Head		3	M	50	Riluzole (100)	67
	Idiopathic	21	Focal	Neck	Head		4	F	42	Riluzole (100)	17
	Idiopathic	13	Focal	Neck	Head		5	F	50	Riluzole (100)	33
33	Idiopathic	3	Focal	Hand	PWT		3	M	73	Trihexyphenidyl (6)	66
82	DRD	15	Multifocal	Neck, vocal cords, hands	Hands	1	1	M	30	Levodopa (300)	0
23	Idiopathic	2	Segmental	Head, jaw	Jaw	3	1	F	61	Trihexyphenidyl, propranolol, imipramine, tetrabenazine, fluvoxamine, sulpiride, benzodiazepines	0
	Idiopathic	29	Segmental	Neck, jaw	Jaw		2	F	76	Trihexyphenidyl, tetrabenazine, clonazepam, baclofen, amitriptyline	0
	Idiopathic	5	Segmental	Head, face	Jaw		4	F	69	Diazepam, tetrabenazine	Improvement
64	Acquired (perinatal anoxia)	14	Multifocal	Neck, arms, foot	Head, arm	1	1	M	28	Bilateral STN DBS	64,9
39	DRD	?	Generalised		Arms	22	?	?	?	Levodopa	Excellent response
Botulinum neurotoxin injections
43	Idiopathic	?	Focal	Neck	Head	17	?	?	?	BoNT-A (50–100 U onabotulinumtoxinA)	65†
48	Idiopathic	13.9‡	Focal	Neck	Head	13	?	?	55.8‡	BoNT-A (107§)	107
48	Idiopathic	13.9‡	Focal	Hand	Hand	1	?	?	55.8‡	BoNT-A (95¶)	95
44	Idiopathic	9.5	Focal	Neck	Head	34	?	15 M/19 F	50.3	BoNT	39.3
45	Idiopathic	8.7	Focal	Neck	Head	29	?	6M/23F	53.8	BoNT-A (500 U abobotulinumtoxinA)	49
47	Idiopathic	?	Focal	Vocal cords	Voice	23	?	?	?	BoNT-A (0.25–2.5 U onabotulinumtoxinA)	12**
47	Idiopathic	?	Focal	Vocal cords	Voice	21	?	?	?	BoNT-A (0.25–2.5 U onabotulinumtoxinA)	13.1**
33	Idiopathic	7	Focal	Hand	PWT	5	1	M	65	BoNT-A (12.5 U onabotulinumtoxinA)	100
	Idiopathic	10	Multifocal	Hands	PWT		2	F	43	BoNT-A (25 U onabotulinumtoxinA)	66
	Idiopathic	3	Focal	Hand	PWT		4	F	48	BoNT-A (30 U onabotulinumtoxinA)	66
	Idiopathic	8	Focal	Hand	PWT		5	M	38	BoNT-A (10 U onabotulinumtoxinA)	66
23	Idiopathic	4	Segmental	Jaw, neck, arms	Jaw, head, arms	1	3	F	78	BoNT	Improvement
46	?	?	?	Head	Head	186	?	31.5%M/68.5%F††	51.9±12.7††	BoNT (500 U abobotulinumtoxinA)	45
CNS surgery
54	Acquired (perinatal anoxia)	23	Generalised		Arm	2	1	M	23	Thalamotomy+Unilateral Vim DBS	Improvement
54	Acquired (perinatal anoxia)	21	Generalised		Arm		2	F	21	Thalamotomy+Unilateral Vim DBS	Improvement
69	Idiopathic	24	Focal	Hand	PWT	1	1	M	40	Unilateral Vim DBS	85.2
66	Idiopathic	37	Multifocal	Arms	Arms	1	1	F	52	Bilateral PSA DBS	Remarkably reduced
55	Idiopathic	33	Focal	Hand	PWT	1	1	M	58	Unilateral Vim DBS	94.4
62	Idiopathic	13	Generalised		Arm	4	1	M	55	Unilateral VLp DBS	Improvement
	Idiopathic	19	Generalised		Right limbs		2	M	32	Unilateral VLp DBS	Improvement
	Idiopathic	4	Multifocal		Arms		3	F	53	Bilateral VLp DBS	Improvement
	Idiopathic	14	Generalised		Arm		4	F	46	Unilateral VLp DBS	100
53	Idiopathic	13.5	Focal	Neck	Head	1	1	F	46.5	Bilateral Gpi DBS	0
63	Idiopathic	15	Segmental	Neck, head	Head, arms	1	1	M	54	Bilateral STN DBS	Moderate head tremor suppression, complete arm tremor suppression
59	Idiopathic	?	Generalised			1	1	?	?	Thalamotomy+Unilateral Vim+Gpi DBS	Dramatic improvement
65	Idiopathic	8	Generalised		Limbs	1	1	M	53	Bilateral cZi DBS	70.5
67	Idiopathic	8	Focal	Hand	Hand	2	1	F	38	Bilateral PSA DBS	100
67	Acquired (head injury)	5	Focal	Hand	Hand		2	M	45	Bilateral PSA DBS	100
56	Myoclonus dystonia	?	Multifocal	Arms	Arms	1	1	F	74	Bilateral Vim DBS	100
57	Acquired (head injury)	10	Hemidystonia		Right limbs	1	1	M	14	Thalamotomy	Slight improvement
60	Idiopathic	15	Segmental	Neck, arms, trunk	Trunk	2	1	F	30	Bilateral Gpi+Vim DBS	Marked improvement
60	Acquired (head injury)	11	Segmental	Eyes, jaw, vocal cords neck, trunk	Head		2	F	74	Bilateral Gpi+Vim DBS	Marked improvement
58	Idiopathic	5	Generalised		Head, voice, hands	3	1	F	47	Bilateral Gpi+Vim DBS	45
	Idiopathic	11	Multifocal	Hands, vocal cords	Hands, voice		2	M	36	Unilateral Vim DBS	51.2
	Idiopathic	4	Segmental	Neck, hands	Head, hands		3	F	71	Bilateral Vim DBS	29.2
61	Myoclonus dystonia	10	Segmental	Neck, arm	Head, arm	1	1	M	23	Bilateral Gpi DBS+Unilateral Vim DBS	62
50	Idiopathic	60	Focal	Neck	Head	1	1	F	76	Unilateral Gpi DBS	75
51	Idiopathic	3	Segmental	Eyes, face, neck	Head	1	1	F	64	Unilateral Gpi DBS	100
52	Idiopathic	?	Hemidystonia	?	Right arm	10	1	M	61	Unilateral Vim DBS	88.9
	Idiopathic	?	Hemidystonia	?	Right arm		2	F	52	Unilateral Vim DBS	93.8
	Idiopathic	?	Hemidystonia	?	Right arm		3	F	50	Unilateral Vim DBS	100
	Idiopathic	?	Generalised	?	Arms		4	M	63	Bilateral Vim DBS	88.3
	Idiopathic	?	Segmental	?	Right arm		5	M	28	Bilateral GPi DBS	44.4
	Idiopathic	?	Generalised	?	Arms		6	F	53	Bilateral GPi DBS	60
	Idiopathic	?	Generalised	?	Arms		7	M	13	Bilateral GPi DBS	50
	Idiopathic	?	Generalised	?	Arms		8	F	74	Bilateral GPi DBS	53.3
	Idiopathic	?	Generalised	?	Arms		9	F	54	Bilateral GPi DBS+unilateral Vim DBS	78.9
	Idiopathic	?	Generalised	?	Arms		10	M	23	Thalamotomy+bilateral GPi DBS+unilateral Vim DBS	70.6
70	Idiopathic	6	Focal	Right hand	PWT	1	1	F	66	Unilateral Vim DBS	100
Other treatments
71	Idiopathic	?	Segmental	Arms, neck	Limbs	1	1	M	34	TENS	Relief of tremor
72	Idiopathic and acquired	?	Focal	Neck	Head	27	?	?	?	SPD	8
75	Idiopathic	9.3	Focal	Hand	PWT	9	?	8M/1F	?	Writing device	136
74	Idiopathic	?	Focal	Hand	PWT	9	?	M	61.6	TENS (5 Hz) flexor carpi	7.7
	Idiopathic	?	Focal	Hand	PWT	9	?	?	?	TENS (25 Hz) flexor carpi	20.4
	Idiopathic	?	Focal	Hand	PWT	9	?	?	?	TENS (50 Hz) flexor carpi	Worsened by 24.3% (p<0.05)
73	Idiopathic	1	Focal	Lower jaw	Jaw	1	1	F	28	Dental splint	100

*Dose and drug not specified.
†The outcome is the percentage of treatment responders.
‡The outcome is the benefit duration (weeks).
§The value refers to the total sample of 51 patients enrolled in this study (14 of them had dystonic tremor).
¶Average dose injected in the total sample of patients with head tremor (not only dystonic tremors).
**Average dose injected in the total sample of patients with hand tremor (not only dystonic tremors).
††Value refers to the total sample of 515 patients enrolled in this study (186 of them had dystonic tremor).
BoNT, botulinum neurotoxin; cZi, caudal zona incerta; DBS, deep brain stimulation, DRD, dopa-responsive dystonia; F, female; Gpi, globus pallidus internus; M, male; PSA, posterior subthalamic area; PWT, primary writing tremor; SPD, selective peripheral denervation; STN, subthalamus; TENS, transcutaneous electrical nerve stimulation; Vim, ventralis intermedius nucleus of thalamus; VLp, posterior part of the ventrolateral thalamus.
CNS, central nervous system.