DBS in pediatric patients: institutional experience
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DBS is initially used for treatment of essential tremor and Parkinson’s disease in adults. In 1996, a child with severe life-threatening dystonia was offered DBS to the internal globus pallidus (GPi) with lasting efficacy at 20 years. Since that time, increasing number of children benefited from DBS.
Patients and methods
We retrospectively evaluated our database of patients who underwent DBS from 2011 to 2017. All patients ≤ 17 years of age at the time of implantation of DBS were included in this series. Subjective Benefit Rating Scale (SBRS), Hoehn Yahr Scale (HYS), Fahn Marsden Rating Scale (FMRS), Clinical Global Impressions Scales (CGI), and Yale Global Tic Severity Scale (YGT) were used to evaluate clinical outcome.
Between May 2014 and October 2017, 11 children underwent DBS procedure in our institution. Six of them were female and five of them were male. Mean age at surgery was 11.8 ± 4.06 years (range 5–17 years). In our series, four patients had primary dystonia (PDY) (36.3%), three patients had secondary dystonia (SDY) (27.2%), two patients had JP (18.1%), and two patients had Tourette Syndrome (TS) (18.1%). Two JP patients underwent bilateral STN DBS while the other nine patients underwent bilateral GPi DBS. SBRS scores were 1.75 ± 0.5 for patients with PDY, 3 ± 0 for patients with JP, 2.5 ± 0.7 for patients with TS, and 2 ± 1 for patients with SDY. Mean FMRS reduction rate was 40.5 for patients with dystonia. Significant improvement was also defined in patients with TS and JP after DBS. None of the patients experienced any intracerebral hemorrhage or other serious adverse neurological effect related to the DBS. Wound complications occurred in two patients.
There are many literatures that support DBS as a treatment option for pediatric patients with medically refractory neurological disorders. DBS has replaced ablative procedures as a treatment of choice not only for adult patients, but also for pediatric patients. Wound-related complications still remain the most common problem in pediatric patients. Development of smaller and more flexible hardware will improve quality of children’s life and minimize wound-related complications in the future.
KeywordsDeep brain stimulation Dystonia GPi Juvenile parkinsonism STN Tourette syndrome
attention-deficit hyperactivity disorder
Clinical Global Impressions Scales
centromedian–parafascicular nuclear complex of the thalamus
deep brain stimulation
Fahn Marsden Rating Scale
internal globus pallidus
Hoehn Yahr Scale
intensive care unit
mitochondrial membrane protein-associated neurodegeneration
magnetic resonance imaging
neurodegeneration with brain iron accumulation
obsessive compulsive disorder
primary generalized dystonia
PTEN-induced putative kinase 1
Subjective Benefit Rating Scale
Yale Global Tic Severity Scale
Compliance with ethical standards
This study was approved by the institutional review board of Istanbul Bilim University, Istanbul, Turkey.
Conflict of interest
There are no conflicts of interest.
- 3.Olaya JE, Christian E, Ferman D, Luc Q, Krieger MD, Sanger TD, Liker MA (2013) Deep brain stimulation in children and young adults with secondary dystonia: the Children’s Hospital Los Angeles experience. Neurosurg Focus 35(5):E7. https://doi.org/10.3171/2013.8.FOCUS13300 CrossRefPubMedGoogle Scholar
- 6.Leckman JF, Riddle MA, Hardin MT, Ort SI, Swartz KL, Stevenson J, Cohen DJ (1989) The Yale Global Tic Severity Scale: initial testing of a clinician-rated scale of tic severity. J Am Acad Child Adolesc Psychiatry 28(4):566–573. https://doi.org/10.1097/00004583-198907000-00015 CrossRefPubMedGoogle Scholar
- 7.DiFrancesco MF, Halpern CH, Hurtig HH, Baltuch GH, Heuer GG (2012) Pediatric indications for deep brain stimulation. Child’s nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery 28(10):1701–1714. https://doi.org/10.1007/s00381-012-1861-2 CrossRefGoogle Scholar
- 9.Bhidayasiri R (2006) Dystonia: genetics and treatment update. Neurologist 12(2):74–85. https://doi.org/10.1097/01.nrl.0000195831.46000.2d CrossRefPubMedGoogle Scholar
- 10.Kupsch A, Benecke R, Muller J, Trottenberg T, Schneider GH, Poewe W, Eisner W, Wolters A, Muller JU, Deuschl G, Pinsker MO, Skogseid IM, Roeste GK, Vollmer-Haase J, Brentrup A, Krause M, Tronnier V, Schnitzler A, Voges J, Nikkhah G, Vesper J, Naumann M, Volkmann J, Deep-Brain Stimulation for Dystonia Study G (2006) Pallidal deep-brain stimulation in primary generalized or segmental dystonia. N Engl J Med 355(19):1978–1990. https://doi.org/10.1056/NEJMoa063618 CrossRefPubMedGoogle Scholar
- 11.Marks WA, Honeycutt J, Acosta F, Jr., Reed M, Bailey L, Pomykal A, Mercer M (2011) Dystonia due to cerebral palsy responds to deep brain stimulation of the globus pallidus internus. Movement disorders : official journal of the Movement Disorder Society 26 (9):1748–1751. doi: https://doi.org/10.1002/mds.23723
- 13.Lobel U, Schweser F, Nickel M, Deistung A, Grosse R, Hagel C, Fiehler J, Schulz A, Hartig M, Reichenbach JR, Kohlschutter A, Sedlacik J (2014) Brain iron quantification by MRI in mitochondrial membrane protein-associated neurodegeneration under iron-chelating therapy. Annals of clinical and translational neurology 1(12):1041–1046. https://doi.org/10.1002/acn3.116 CrossRefPubMedPubMedCentralGoogle Scholar
- 14.Yilmaz S, Gokben S, Ceylaner S (2015) Mitochondrial membrane protein-associated neurodegeneration. Pediatr Neurol 53(4):373–374. https://doi.org/10.1016/j.pediatrneurol.2015.06.012 CrossRefPubMedGoogle Scholar
- 18.Schrock LE, Mink JW, Woods DW, Porta M, Servello D, Visser-Vandewalle V, Silburn PA, Foltynie T, Walker HC, Shahed-Jimenez J, Savica R, Klassen BT, Machado AG, Foote KD, Zhang JG, Hu W, Ackermans L, Temel Y, Mari Z, Changizi BK, Lozano A, Auyeung M, Kaido T, Agid Y, Welter ML, Khandhar SM, Mogilner AY, Pourfar MH, Walter BL, Juncos JL, Gross RE, Kuhn J, Leckman JF, Neimat JA, Okun MS, Tourette Syndrome Association International Deep Brain Stimulation D, Registry Study G (2015) Tourette syndrome deep brain stimulation: a review and updated recommendations. Movement disorders : official journal of the Movement Disorder Society 30(4):448–471. https://doi.org/10.1002/mds.26094 CrossRefGoogle Scholar
- 20.Genc G, Apaydin H, Gunduz A, Poyraz C, Oguz S, Yagci S, Canaz H, Aydin S, Gundogdu-Eken A, Basak AN, Ertan S (2016) Successful treatment of juvenile parkinsonism with bilateral subthalamic deep brain stimulation in a 14-year-old patient with parkin gene mutation. Parkinsonism Relat Disord 24:137–138. https://doi.org/10.1016/j.parkreldis.2016.01.018 CrossRefPubMedGoogle Scholar
- 21.Courchesne E, Chisum HJ, Townsend J, Cowles A, Covington J, Egaas B, Harwood M, Hinds S, Press GA (2000) Normal brain development and aging: quantitative analysis at in vivo MR imaging in healthy volunteers. Radiology 216(3):672–682. https://doi.org/10.1148/radiology.216.3.r00au37672 CrossRefPubMedPubMedCentralGoogle Scholar