Skip to main content
Top
Published in: Child's Nervous System 3/2024

25-10-2023 | Dystonia | Original Article

Deep brain stimulation in pediatric dystonia: calls for therapeutic realism over nihilism

Authors: Souvik Singha, Srinivas Dwarakanath, Ravi Yadav, Vikram V. Holla, Nitish Kamble, Gaurav Tyagi, Pramod Kumar Pal

Published in: Child's Nervous System | Issue 3/2024

Login to get access

Abstract

Purpose

Pediatric dystonia (PD) has a significant negative impact on the growth and development of the child. This study was done retrospectively to analyze functional outcomes in pediatric patients with dystonia who underwent deep brain stimulation.

Methods

In this retrospective analytical study, all the patients of age less than 18 years undergoing deep brain stimulation (DBS) for dystonia between 2012 and 2020 in a single center were analyzed and their functional outcomes were measured by the Burke–Fahn–Marsden-dystonia-rating-scale (BFMDRS).

Results

A total of 10 pediatric patients were included with a mean age of onset, duration of disease, and age at surgery being 5.75 years, 7.36 years, and 13.11 years, respectively, with a mean follow-up of 23.22 months. The mean pre-DBS motor score was 75.44 ± 23.53 which improved significantly at 6-month and 12-month follow-up to 57.27 (p value 0.004) and 50.38 (p value < 0.001), respectively. Limbs sub-scores improved significantly at both the scheduled intervals. There was a significant improvement in disability at 1-year follow-up with significant improvement in feeding, dressing, and walking components. There was a 27.34% and 36.64% improvement in dystonia with a 17.37% and 28.86% reduction in disability at 6 months and 12 months, respectively. There was a positive correlation between the absolute reduction of the motor score and improvement in disability of the patients at 6 months (rho = 0.865, p value 0.003).

Conclusions

DBS in PD has an enormous role in reducing disease burden and achieving a sustainable therapeutic goal.
Literature
1.
go back to reference Mink JW (2013) Special concerns in defining, studying, and treating dystonia in children. Mov Disord 28:921–925PubMedCrossRef Mink JW (2013) Special concerns in defining, studying, and treating dystonia in children. Mov Disord 28:921–925PubMedCrossRef
2.
go back to reference de Ligt J et al (2012) Diagnostic exome sequencing in persons with severe intellectual disability. N Engl J Med 367:1921–1929PubMedCrossRef de Ligt J et al (2012) Diagnostic exome sequencing in persons with severe intellectual disability. N Engl J Med 367:1921–1929PubMedCrossRef
3.
go back to reference García-Cazorla A et al (2009) Inborn errors of metabolism and motor disturbances in children. J Inherit Metab Dis 32:618–629PubMedCrossRef García-Cazorla A et al (2009) Inborn errors of metabolism and motor disturbances in children. J Inherit Metab Dis 32:618–629PubMedCrossRef
4.
go back to reference Albanese A et al (2013) Phenomenology and classification of dystonia: a consensus update. Mov Disord 28:863–873PubMedCrossRef Albanese A et al (2013) Phenomenology and classification of dystonia: a consensus update. Mov Disord 28:863–873PubMedCrossRef
5.
go back to reference van Egmond ME et al (2015) Dystonia in children and adolescents: a systematic review and a new diagnostic algorithm. J Neurol Neurosurg Psych 86:774–781PubMedCrossRef van Egmond ME et al (2015) Dystonia in children and adolescents: a systematic review and a new diagnostic algorithm. J Neurol Neurosurg Psych 86:774–781PubMedCrossRef
6.
7.
go back to reference Kojovic M et al (2013) Secondary and primary dystonia: pathophysiological differences. Brain : J Neurol 136:2038–2049PubMedCrossRef Kojovic M et al (2013) Secondary and primary dystonia: pathophysiological differences. Brain : J Neurol 136:2038–2049PubMedCrossRef
10.
go back to reference Sanger TD et al (2010) Definition and classification of hyperkinetic movements in childhood. Mov Disord 25:1538–1549PubMedCrossRef Sanger TD et al (2010) Definition and classification of hyperkinetic movements in childhood. Mov Disord 25:1538–1549PubMedCrossRef
11.
go back to reference Ozturk S, Temel Y, Aygun D, Kocabicak E (2021) Deep brain stimulation of the Globus Pallidus Internus for secondary dystonia: clinical cases and systematic review of the literature regarding the effectiveness of Globus Pallidus Internus versus subthalamic nucleus. World Neurosurg 154:e495–e508PubMedCrossRef Ozturk S, Temel Y, Aygun D, Kocabicak E (2021) Deep brain stimulation of the Globus Pallidus Internus for secondary dystonia: clinical cases and systematic review of the literature regarding the effectiveness of Globus Pallidus Internus versus subthalamic nucleus. World Neurosurg 154:e495–e508PubMedCrossRef
12.
go back to reference Mundinger F (1977) New stereotactic treatment of spasmodic torticollis with a brain stimulation system (author’s transl). Med Klin 72:1982–1986PubMed Mundinger F (1977) New stereotactic treatment of spasmodic torticollis with a brain stimulation system (author’s transl). Med Klin 72:1982–1986PubMed
13.
go back to reference Benabid AL, Pollak P, Louveau A, Henry S, de Rougemont J (1987) Combined (thalamotomy and stimulation) stereotactic surgery of the VIM thalamic nucleus for bilateral Parkinson disease. Appl Neurophysiol 50:344–346PubMed Benabid AL, Pollak P, Louveau A, Henry S, de Rougemont J (1987) Combined (thalamotomy and stimulation) stereotactic surgery of the VIM thalamic nucleus for bilateral Parkinson disease. Appl Neurophysiol 50:344–346PubMed
14.
go back to reference Marks WA, Honeycutt J, Acosta F, Reed M (2009) Deep brain stimulation for pediatric movement disorders. Semin Pediatr Neurol 16:90–98PubMedCrossRef Marks WA, Honeycutt J, Acosta F, Reed M (2009) Deep brain stimulation for pediatric movement disorders. Semin Pediatr Neurol 16:90–98PubMedCrossRef
15.
go back to reference Coubes P et al (1999) Treatment of early-onset generalized dystonia by chronic bilateral stimulation of the internal globus pallidus. Apropos of a case. Neuro-Chirurgie 45:139–144 Coubes P et al (1999) Treatment of early-onset generalized dystonia by chronic bilateral stimulation of the internal globus pallidus. Apropos of a case. Neuro-Chirurgie 45:139–144
16.
go back to reference Cif L, Coubes P (2017) Historical developments in children’s deep brain stimulation. Eur J Paediatr Neurol Eur J Paediatr Nuero 21:109–117PubMedCrossRef Cif L, Coubes P (2017) Historical developments in children’s deep brain stimulation. Eur J Paediatr Neurol Eur J Paediatr Nuero 21:109–117PubMedCrossRef
17.
go back to reference Air EL, Ostrem JL, Sanger TD, Starr PA (2011) Deep brain stimulation in children: experience and technical pearls. J Neurosurg Pediatr 8:566–574PubMedCrossRef Air EL, Ostrem JL, Sanger TD, Starr PA (2011) Deep brain stimulation in children: experience and technical pearls. J Neurosurg Pediatr 8:566–574PubMedCrossRef
18.
go back to reference Lumsden DE et al (2013) Proportion of life lived with dystonia inversely correlates with response to pallidal deep brain stimulation in both primary and secondary childhood dystonia. Dev Med Child Neurol 55:567–574PubMedCrossRef Lumsden DE et al (2013) Proportion of life lived with dystonia inversely correlates with response to pallidal deep brain stimulation in both primary and secondary childhood dystonia. Dev Med Child Neurol 55:567–574PubMedCrossRef
19.
go back to reference Olaya JE et al (2013) Deep brain stimulation in children and young adults with secondary dystonia: the Children’s Hospital Los Angeles experience. Neurosurg Focus 35:E7PubMedCrossRef Olaya JE et al (2013) Deep brain stimulation in children and young adults with secondary dystonia: the Children’s Hospital Los Angeles experience. Neurosurg Focus 35:E7PubMedCrossRef
20.
go back to reference Sanger TD et al (2018) Pediatric deep brain stimulation using awake recording and stimulation for target selection in an inpatient neuromodulation monitoring unit. Brain Sci 8 Sanger TD et al (2018) Pediatric deep brain stimulation using awake recording and stimulation for target selection in an inpatient neuromodulation monitoring unit. Brain Sci 8
21.
go back to reference Cersosimo MG et al (2008) Pallidal surgery for the treatment of primary generalized dystonia: long-term follow-up. Clin Neurol Neurosurg 110:145–150PubMedCrossRef Cersosimo MG et al (2008) Pallidal surgery for the treatment of primary generalized dystonia: long-term follow-up. Clin Neurol Neurosurg 110:145–150PubMedCrossRef
22.
go back to reference Ghosh PS, Machado AG, Deogaonkar M, Ghosh D (2012) Deep brain stimulation in children with dystonia: experience from a tertiary care center. Pediatr Neurosurg 48:146–151PubMedCrossRef Ghosh PS, Machado AG, Deogaonkar M, Ghosh D (2012) Deep brain stimulation in children with dystonia: experience from a tertiary care center. Pediatr Neurosurg 48:146–151PubMedCrossRef
23.
go back to reference Goto S et al (2006) Impact of bilateral pallidal stimulation on DYT1-generalized dystonia in Japanese patients. Mov Disord 21:1785–1787PubMedCrossRef Goto S et al (2006) Impact of bilateral pallidal stimulation on DYT1-generalized dystonia in Japanese patients. Mov Disord 21:1785–1787PubMedCrossRef
25.
go back to reference Keen JR, Przekop A, Olaya JE, Zouros A, Hsu FPK (2014) Deep brain stimulation for the treatment of childhood dystonic cerebral palsy. J Neurosurg Pediatr 14:585–593PubMedCrossRef Keen JR, Przekop A, Olaya JE, Zouros A, Hsu FPK (2014) Deep brain stimulation for the treatment of childhood dystonic cerebral palsy. J Neurosurg Pediatr 14:585–593PubMedCrossRef
27.
go back to reference Krause P et al (2016) Long-term results of deep brain stimulation in a cohort of eight children with isolated dystonia. J Neurol 263:2319–2326PubMedCrossRef Krause P et al (2016) Long-term results of deep brain stimulation in a cohort of eight children with isolated dystonia. J Neurol 263:2319–2326PubMedCrossRef
28.
go back to reference Kupsch A et al (2003) The effects of frequency in pallidal deep brain stimulation for primary dystonia. J Neurol 250:1201–1205PubMedCrossRef Kupsch A et al (2003) The effects of frequency in pallidal deep brain stimulation for primary dystonia. J Neurol 250:1201–1205PubMedCrossRef
29.
go back to reference Mehrkens JH, Borggraefe I, Feddersen B, Heinen F, Bötzel K (2010) Early globus pallidus internus stimulation in pediatric patients with generalized primary dystonia: long-term efficacy and safety. J Child Neurol 25:1355–1361PubMedCrossRef Mehrkens JH, Borggraefe I, Feddersen B, Heinen F, Bötzel K (2010) Early globus pallidus internus stimulation in pediatric patients with generalized primary dystonia: long-term efficacy and safety. J Child Neurol 25:1355–1361PubMedCrossRef
30.
go back to reference Miyagi Y, Koike Y (2013) Tolerance of early pallidal stimulation in pediatric generalized dystonia. J Neurosurg Pediatr 12:476–482PubMedCrossRef Miyagi Y, Koike Y (2013) Tolerance of early pallidal stimulation in pediatric generalized dystonia. J Neurosurg Pediatr 12:476–482PubMedCrossRef
31.
go back to reference Oterdoom DLM et al (2018) Reversal of status dystonicus after relocation of pallidal electrodes in DYT6 generalized dystonia. Tremor and Other Hyperkinetic Movements (New York, N.Y.) 8:530 Oterdoom DLM et al (2018) Reversal of status dystonicus after relocation of pallidal electrodes in DYT6 generalized dystonia. Tremor and Other Hyperkinetic Movements (New York, N.Y.) 8:530
32.
go back to reference Parr JR et al (2007) Deep brain stimulation in childhood: an effective treatment for early onset idiopathic generalised dystonia. Arch Dis Child 92:708–711PubMedPubMedCentralCrossRef Parr JR et al (2007) Deep brain stimulation in childhood: an effective treatment for early onset idiopathic generalised dystonia. Arch Dis Child 92:708–711PubMedPubMedCentralCrossRef
34.
go back to reference Starr PA et al (2004) Microelectrode-guided implantation of deep brain stimulators into the globus pallidus internus for dystonia: techniques, electrode locations, and outcomes. Neurosurg Focus 17:E4PubMedCrossRef Starr PA et al (2004) Microelectrode-guided implantation of deep brain stimulators into the globus pallidus internus for dystonia: techniques, electrode locations, and outcomes. Neurosurg Focus 17:E4PubMedCrossRef
35.
go back to reference Tronnier VM, Fogel W (2000) Pallidal stimulation for generalized dystonia. Report of three cases. J Neurosurg 92:453–456 Tronnier VM, Fogel W (2000) Pallidal stimulation for generalized dystonia. Report of three cases. J Neurosurg 92:453–456
36.
go back to reference Vidailhet M et al (2005) Bilateral deep-brain stimulation of the globus pallidus in primary generalized dystonia. N Engl J Med 352:459–467PubMedCrossRef Vidailhet M et al (2005) Bilateral deep-brain stimulation of the globus pallidus in primary generalized dystonia. N Engl J Med 352:459–467PubMedCrossRef
37.
go back to reference Zorzi G et al (2005) Stimulation of the globus pallidus internus for childhood-onset dystonia. Mov Disord 20:1194–1200PubMedCrossRef Zorzi G et al (2005) Stimulation of the globus pallidus internus for childhood-onset dystonia. Mov Disord 20:1194–1200PubMedCrossRef
38.
go back to reference Krause P et al (2015) Long-term effect on dystonia after pallidal deep brain stimulation (DBS) in three members of a family with a THAP1 mutation. J Neurol 262:2739–2744PubMedCrossRef Krause P et al (2015) Long-term effect on dystonia after pallidal deep brain stimulation (DBS) in three members of a family with a THAP1 mutation. J Neurol 262:2739–2744PubMedCrossRef
39.
go back to reference San Luciano M et al (2020) Thalamic deep brain stimulation for acquired dystonia in children and young adults: a phase 1 clinical trial. J Neurosurg Pediatr 27:203–212 San Luciano M et al (2020) Thalamic deep brain stimulation for acquired dystonia in children and young adults: a phase 1 clinical trial. J Neurosurg Pediatr 27:203–212
40.
go back to reference Arumugham SS et al (2021) Identification of biomarkers that predict response to subthalamic nucleus deep brain stimulation in resistant obsessive-compulsive disorder: protocol for an open-label follow-up study. BMJ Open 11:e047492PubMedPubMedCentralCrossRef Arumugham SS et al (2021) Identification of biomarkers that predict response to subthalamic nucleus deep brain stimulation in resistant obsessive-compulsive disorder: protocol for an open-label follow-up study. BMJ Open 11:e047492PubMedPubMedCentralCrossRef
41.
go back to reference Manjunath M et al (2017) Experience of pallidal deep brain stimulation in dystonia at a tertiary care centre in India: An initial experience. Neurol India 65:1322–1329PubMedCrossRef Manjunath M et al (2017) Experience of pallidal deep brain stimulation in dystonia at a tertiary care centre in India: An initial experience. Neurol India 65:1322–1329PubMedCrossRef
42.
go back to reference Larsh T, Wu SW, Vadivelu S, Grant GA, O’Malley JA (2021) Deep brain stimulation for pediatric dystonia. Seminars in pediatric neurology 38:100896PubMedCrossRef Larsh T, Wu SW, Vadivelu S, Grant GA, O’Malley JA (2021) Deep brain stimulation for pediatric dystonia. Seminars in pediatric neurology 38:100896PubMedCrossRef
43.
go back to reference Andrews C, Aviles-Olmos I, Hariz M, Foltynie T (2010) Which patients with dystonia benefit from deep brain stimulation? A metaregression of individual patient outcomes. J Neurol Neurosurg Psychiatry 81:1383–1389PubMedCrossRef Andrews C, Aviles-Olmos I, Hariz M, Foltynie T (2010) Which patients with dystonia benefit from deep brain stimulation? A metaregression of individual patient outcomes. J Neurol Neurosurg Psychiatry 81:1383–1389PubMedCrossRef
44.
go back to reference Panov F et al (2023) Deep brain stimulation in DYT1 dystonia: a 10-year experience. Neurosurgery 73:86–93; discussion 93 Panov F et al (2023) Deep brain stimulation in DYT1 dystonia: a 10-year experience. Neurosurgery 73:86–93; discussion 93
45.
go back to reference De Vloo P et al (2019) Deep brain stimulation for pantothenate kinase-associated neurodegeneration: a meta-analysis. Mov Disord 34:264–273PubMedCrossRef De Vloo P et al (2019) Deep brain stimulation for pantothenate kinase-associated neurodegeneration: a meta-analysis. Mov Disord 34:264–273PubMedCrossRef
46.
go back to reference Sidiropoulos C et al (2013) Bilateral pallidal stimulation for Wilson’s disease. Mov Disord 28:1292–1295PubMedCrossRef Sidiropoulos C et al (2013) Bilateral pallidal stimulation for Wilson’s disease. Mov Disord 28:1292–1295PubMedCrossRef
47.
go back to reference Fan H, Zheng Z, Yin Z, Zhang J, Lu G (2021) Deep brain stimulation treating dystonia: a systematic review of targets, body distributions and etiology classifications. Front Hum Neurosci 15:757579PubMedPubMedCentralCrossRef Fan H, Zheng Z, Yin Z, Zhang J, Lu G (2021) Deep brain stimulation treating dystonia: a systematic review of targets, body distributions and etiology classifications. Front Hum Neurosci 15:757579PubMedPubMedCentralCrossRef
48.
go back to reference van Riesen C et al (2016) Disease-specific longevity of impulse generators in deep brain stimulation and review of the literature. J Neural Trans (Vienna, Austria : 1996) 123:621–630 van Riesen C et al (2016) Disease-specific longevity of impulse generators in deep brain stimulation and review of the literature. J Neural Trans (Vienna, Austria : 1996) 123:621–630
49.
go back to reference Berman BD, Starr PA, Marks WJJ, Ostrem JL (2009) Induction of bradykinesia with pallidal deep brain stimulation in patients with cranial-cervical dystonia. Stereotact Funct Neurosurg 87:37–44PubMedPubMedCentralCrossRef Berman BD, Starr PA, Marks WJJ, Ostrem JL (2009) Induction of bradykinesia with pallidal deep brain stimulation in patients with cranial-cervical dystonia. Stereotact Funct Neurosurg 87:37–44PubMedPubMedCentralCrossRef
50.
go back to reference Schrader C et al (2011) GPi-DBS may induce a hypokinetic gait disorder with freezing of gait in patients with dystonia. Neurology 77:483–488PubMedCrossRef Schrader C et al (2011) GPi-DBS may induce a hypokinetic gait disorder with freezing of gait in patients with dystonia. Neurology 77:483–488PubMedCrossRef
51.
go back to reference Ostrem JL et al (2017) Subthalamic nucleus deep brain stimulation in isolated dystonia: a 3-year follow-up study. Neurology 88:25–35PubMedCrossRef Ostrem JL et al (2017) Subthalamic nucleus deep brain stimulation in isolated dystonia: a 3-year follow-up study. Neurology 88:25–35PubMedCrossRef
52.
go back to reference Schjerling L et al (2013) A randomized double-blind crossover trial comparing subthalamic and pallidal deep brain stimulation for dystonia. J Neurosurg 119:1537–1545PubMedCrossRef Schjerling L et al (2013) A randomized double-blind crossover trial comparing subthalamic and pallidal deep brain stimulation for dystonia. J Neurosurg 119:1537–1545PubMedCrossRef
53.
go back to reference Lin S et al (2019) Deep brain stimulation of the globus pallidus internus versus the subthalamic nucleus in isolated dystonia. J Neurosurg 132:721–732PubMedCrossRef Lin S et al (2019) Deep brain stimulation of the globus pallidus internus versus the subthalamic nucleus in isolated dystonia. J Neurosurg 132:721–732PubMedCrossRef
54.
go back to reference Ruge D et al (2011) Shaping reversibility? Long-term deep brain stimulation in dystonia: the relationship between effects on electrophysiology and clinical symptoms. Brain : J Neurol 134:2106–2115PubMedCrossRef Ruge D et al (2011) Shaping reversibility? Long-term deep brain stimulation in dystonia: the relationship between effects on electrophysiology and clinical symptoms. Brain : J Neurol 134:2106–2115PubMedCrossRef
55.
go back to reference Elkaim LM et al (2019) Deep brain stimulation for pediatric dystonia: a meta-analysis with individual participant data. Dev Med Child Neurol 61:49–56PubMedCrossRef Elkaim LM et al (2019) Deep brain stimulation for pediatric dystonia: a meta-analysis with individual participant data. Dev Med Child Neurol 61:49–56PubMedCrossRef
56.
go back to reference Hale AT, Monsour MA, Rolston JD, Naftel RP, Englot DJ (2020) Deep brain stimulation in pediatric dystonia: a systematic review. Neurosurg Rev 43:873–880PubMedCrossRef Hale AT, Monsour MA, Rolston JD, Naftel RP, Englot DJ (2020) Deep brain stimulation in pediatric dystonia: a systematic review. Neurosurg Rev 43:873–880PubMedCrossRef
57.
go back to reference Koy A et al (2019) Adverse events associated with deep brain stimulation in patients with childhood-onset dystonia. Brain Stimul 12:1111–1120PubMedCrossRef Koy A et al (2019) Adverse events associated with deep brain stimulation in patients with childhood-onset dystonia. Brain Stimul 12:1111–1120PubMedCrossRef
Metadata
Title
Deep brain stimulation in pediatric dystonia: calls for therapeutic realism over nihilism
Authors
Souvik Singha
Srinivas Dwarakanath
Ravi Yadav
Vikram V. Holla
Nitish Kamble
Gaurav Tyagi
Pramod Kumar Pal
Publication date
25-10-2023
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System / Issue 3/2024
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-023-06182-x

Other articles of this Issue 3/2024

Child's Nervous System 3/2024 Go to the issue