Deep brain stimulation in children: experience and technical pearls

Air, Ellen L.; Ostrem, Jill L.; Sanger, Terence D.; Starr, Philip A.

doi:10.3171/2011.8.peds11153

Cited by 132 publications

(142 citation statements)

References 45 publications

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“…1,3,13,23,24,26,42 Cerebral palsy is the most common cause of secondary dystonia. 33 The incidence of cerebral palsy is approximately 2 per 1000 births.…”

Section: Discussionmentioning

confidence: 99%

“…The most commonly used scale has been the Burke-FahnMarsden Dystonia Rating Scale (BFMDRS); 7 however, some authors have also used a modified BFMDRS known as the Barry-Albright Dystonia Scale (BADS). 1,24,26 This scale was specifically developed to assess patients with secondary dystonia. 4 The BFMDRS assesses movement primarily related to function, but many patients with secondary dystonia have significant cognitive impairments, making it difficult to assess their voluntary control of movements and ultimate functional capacity.…”

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confidence: 99%

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Deep brain stimulation in children and young adults with secondary dystonia: the Children's Hospital Los Angeles experience

Olaya

Christian

Ferman

et al. 2013

FOC

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Background Dystonia is a movement disorder in which involuntary sustained or intermittent muscle contractions cause twisting and repetitive movements, abnormal postures, or both. It can be classified as primary or secondary. There is no cure for dystonia and the goal of treatment is to provide a better quality of life for the patient. Surgical intervention is considered for patients in whom an adequate trial of medical treatment has failed. Deep brain stimulation (DBS), specifically of the globus pallidus interna (GPi), has been shown to be extremely effective in primary generalized dystonia. There is much less evidence for the use of DBS in patients with secondary dystonia. However, given the large number of patients with secondary dystonia, the significant burden on the patients and their families, and the potential for DBS to improve their functional status and comfort level, it is important to continue to investigate the use of DBS in the realm of secondary dystonia. Object The objective of this study is to review a series of cases involving patients with secondary dystonia who have been treated with pallidal DBS. Methods A retrospective review of 9 patients with secondary dystonia who received treatment with DBS between February 2011 and February 2013 was performed. Preoperative and postoperative videos were scored using the Barry-Albright Dystonia Scale (BADS) and Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) by a neurologist specializing in movement disorders. In addition, the patients' families completed a subjective questionnaire to assess the perceived benefit of DBS. Results The average age at DBS unit implantation was 15.1 years (range 6–20 years). The average time to follow-up for the BADS evaluation from battery implantation was 3.8 months (median 3 months). The average time to follow-up for the subjective benefit evaluation was 10.6 months (median 9.5 months). The mean BADS scores improved by 9% from 26.5 to 24 (p = 0.04), and the mean BFMDRS scores improved by 9.3% (p = 0.055). Of note, even in patients with minimal functional improvement, there seemed to be decreased contractures and spasms leading to improved comfort. There were no complications such as infections or hematoma in this case series. In the subjective benefit evaluation, 3 patients' families reported “good” benefit, 4 reported “minimal” benefit, and 1 reported no benefit. Conclusions These early results of GPi stimulation in a series of 9 patients suggest that DBS is useful in the treatment of secondary generalized dystonia in children and young adults. Objective improvements in BADS and BFMDRS scores are demonstrated in some patients with generalized secondary dystonia but not in others. Larger follow-up studies of DBS for secondary dystonia, focusing on patient age, history, etiology, and patterns of dystonia, are needed to learn which patients will respond best to DBS.

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“…1,3,13,23,24,26,42 Cerebral palsy is the most common cause of secondary dystonia. 33 The incidence of cerebral palsy is approximately 2 per 1000 births.…”

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

Deep brain stimulation in children and young adults with secondary dystonia: the Children's Hospital Los Angeles experience

Olaya

Christian

Ferman

et al. 2013

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“…65 Air and colleagues had 11 pediatric patients with secondary dystonia in their series; however, they only reported outcomes in 4 and their results were more modest than the prior studies. 4 Three patients had a 10% improvement in the BFMDRS motor score and a 20% improvement in the disability score. Zorzi and colleagues reported on 3 patients with secondary dystonia treated with DBS.…”

Section: Treatment Of Dystoniamentioning

confidence: 99%

Deep brain stimulation: a mechanistic and clinical update

Karas

Mikell

Christian

et al. 2013

FOC

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Deep brain stimulation (DBS), the practice of placing electrodes deep into the brain to stimulate subcortical structures with electrical current, has been increasing as a neurosurgical procedure over the past 15 years. Originally a treatment for essential tremor, DBS is now used and under investigation across a wide spectrum of neurological and psychiatric disorders. In addition to applying electrical stimulation for clinical symptomatic relief, the electrodes implanted can also be used to record local electrical activity in the brain, making DBS a useful research tool. Human single-neuron recordings and local field potentials are now often recorded intraoperatively as electrodes are implanted. Thus, the increasing scope of DBS clinical applications is being matched by an increase in investigational use, leading to a rapidly evolving understanding of cortical and subcortical neurocircuitry. In this review, the authors discuss recent innovations in the clinical use of DBS, both in approved indications as well as in indications under investigation. Deep brain stimulation as an investigational tool is also reviewed, paying special attention to evolving models of basal ganglia and cortical function in health and disease. Finally, the authors look to the future across several indications, highlighting gaps in knowledge and possible future directions of DBS treatment.

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“…22 Reports of DBS in children with dystonia have been limited. 1,2,9,32 We report a case of total body dystonia secondary to MMA in a 4-year-old boy, which improved after bilateral STN DBS. The methylmalonic acidemias (MMAs) are a group of inborn errors of metabolism resulting in the accumulation of methylmalonic acid in body tissues and fluids.…”

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confidence: 99%

Bilateral subthalamic nucleus deep brain stimulation for refractory total body dystonia secondary to metabolic autopallidotomy in a 4-year-old boy with infantile methylmalonic acidemia

Chakraborti¹,

Hasegawa²,

Lumsden

et al. 2013

PED

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The methylmalonic acidemias (MMAs) are a group of inborn errors of metabolism resulting in the accumulation of methylmalonic acid in body tissues and fluids. A recognized complication of MMA is bilateral liquefaction of the globus pallidi, resulting in a fulminant total body dystonia of childhood often refractory to medical treatment. This case of total body dystonia due to MMA in a 4-year-old boy had been medically refractory for 15 months. Complete metabolic destructive liquefaction of the pallidi, that is, autopallidotomy, necessitated an alternative, bilateral subthalamic nucleus (STN) target for deep brain stimulation (DBS) with a marked improvement in dystonia and reduction in pain. The case illustrates the efficacy of STN DBS in this condition and the technical challenges in targeting the STN in a small child.

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Deep brain stimulation in children: experience and technical pearls

Cited by 132 publications

References 45 publications

Deep brain stimulation in children and young adults with secondary dystonia: the Children's Hospital Los Angeles experience

Deep brain stimulation in children and young adults with secondary dystonia: the Children's Hospital Los Angeles experience

Deep brain stimulation: a mechanistic and clinical update

Bilateral subthalamic nucleus deep brain stimulation for refractory total body dystonia secondary to metabolic autopallidotomy in a 4-year-old boy with infantile methylmalonic acidemia

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