Deep Brain Stimulation for Pediatric Dystonia: A Review of the Literature and Suggested Programming Algorithm

Gelineau‐Morel, Rose; Kruer, Michael C.; Garris, Jordan; Libdeh, Amal Abu; Barbosa, Daniel A. N.; Coffman, Keith A.; Moon, David; Barton, Christopher; Zea-Vera, Alonso; Bruce, Adrienne; Larsh, Travis; Wu, Steve W.; Gilbert, Donald L.; O’Malley, Jennifer A.

doi:10.1177/08830738221115248

Cited by 12 publications

(10 citation statements)

References 89 publications

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“…The target for DBS in dystonia is typically the globus pallidus internus (GPi). 44 However, in patients with lesion-induced dystonia, their GPi is frequently injured or atrophied, and may not be the ideal site for DBS lead implantation. Others have speculated that cerebellar stimulation may provide an alternative site for DBS in dystonia patients.…”

Section: Discussionmentioning

confidence: 99%

Network localization of pediatric lesion-induced dystonia

Gelineau-Morel,

Dlamini,

Bruss

et al. 2024

Preprint

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Objective: Dystonia is a movement disorder defined by involuntary muscle contractions leading to abnormal postures or twisting and repetitive movements. Classically dystonia has been thought of as a disorder of the basal ganglia, but newer results in idiopathic dystonia and lesion-induced dystonia in adults point to broader motor network dysfunction spanning the basal ganglia, cerebellum, premotor cortex, sensorimotor, and frontoparietal regions. It is unclear whether a similar network is shared between different etiologies of pediatric lesion-induced dystonia. Methods: Three cohorts of pediatric patients with lesion-induced dystonia were identified. The lesion etiologies included hypoxia, kernicterus, and stroke versus comparison subjects with acquired lesions not associated with dystonia. Multivariate lesion-symptom mapping and lesion network mapping were used to evaluate the anatomy and networks associated with dystonia. Results: Multivariate lesion-symptom mapping showed that lesions of the putamen (stroke: r = 0.50, p <0.01; hypoxia, r = 0.64, p <0.001) and globus pallidus (kernicterus, r = 0.61, p <0.01) were associated with dystonia. Lesion network mapping using normative connectome data from healthy children demonstrated that these regional findings occurred within a common brain-wide network that involves the basal ganglia, anterior and medial cerebellum, and cortical regions that overlap the cingulo-opercular and somato-cognitive-action networks. Interpretation: We interpret these findings as novel evidence for a unified dystonia brain network that involves the somato-cognitive-action network, which is involved in higher order coordination of movement. Elucidation of this network gives insight into the functional origins of dystonia and provides novel targets to investigate for therapeutic intervention.

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Section: Discussionmentioning

confidence: 99%

Network localization of pediatric lesion-induced dystonia

Gelineau-Morel,

Dlamini,

Bruss

et al. 2024

Preprint

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“…GPi mapping was performed unilaterally with each contact probed beginning at 0.2 v, 185 Hz, and 90 μs, with gradual increases by 0.1 v to a maximum of 4 v or when side effects were appreciated. Given the neuroanatomy and previous known response to circumferential stimulation in GPi ( Gelineau-Morel et al, 2022 ), segmented contacts were not initially explored for mapping. It has been noted by the clinicians that while the effects of GPi stimulation are best seen longitudinally, initial effects on dystonia could be appreciated by the clinician, patient, and family.…”

Section: Methodsmentioning

confidence: 99%

Novel utilization of deep brain stimulation in the pedunculopontine nucleus with globus pallidus internus for treatment of childhood-onset dystonia

MacLean,

Nataraj,

Davies

et al. 2023

Front. Hum. Neurosci.

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IntroductionDeep brain stimulation (DBS) is a well-documented therapy for dystonia utilized in many adult and pediatric movement disorders. Pedunculopontine nucleus (PPN) has been investigated as a DBS target primarily in adult patients with dystonia or dyskinesias from Parkinson’s disease, showing improvement in postural instability and gait dysfunction. Due to the difficulty in targeting PPN using standard techniques, it is not commonly chosen as a target for adult or pediatric pathology. There is no current literature describing the targeting of PPN in DBS for childhood-onset dystonia.MethodsTwo pediatric and one young adult patient with childhood-onset dystonia who underwent DBS implantation at our institution were identified. Patient 1 has Mitochondrial Enoyl CoA Reductase Protein-Associated Neurodegeneration (MEPAN) syndrome. Patient 2 has Glutaric Aciduria Type 1 (GA1). Patient 3 has atypical pantothenate kinase-associated neurodegeneration (PKAN). PPN was identified as a potential target for these patients due to axial or orofacial dystonia. Pre- and post-operative videos taken as part of routine clinical assessments were evaluated and scored on the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) and Barry-Albright Dystonia Scale (BADS). All patients had permanent electrodes placed bilaterally in PPN and globus pallidus internus (GPi). A Likert scale on quality of life was also obtained from the patient/parents as applicable.ResultsSignificant programming was necessary over the first 3–12 months to optimize patients’ response to stimulation. All patients experienced at least a 34% improvement in the BFMDRS score. Patients 2 and 3 also experienced an over 30% improvement in BADS score. All patients/parents appreciated improvement in quality of life postoperatively.DiscussionDeep brain stimulation in PPN was safely and successfully used in two pediatric patients and one young adult patient with childhood-onset dystonia. These patients showed clinically significant improvements in BFMDRS scoring post operatively. This represents the first reported DBS targeting of PPN in pediatric patients, and suggests that PPN is a possible target for pediatric-onset dystonia with axial and orofacial symptoms that may be refractory to traditional pallidal stimulation alone.

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“…Intraoperative visualization of brain anatomy and site targeting require significantly increased precision to successfully localize DBS electrodes[ 75 , 76 ]. Furthermore, children may lack the appropriate endurance and communication abilities needed to determine the therapeutic window during the postoperative stimulation sessions[ 77 ]. Therefore, reaching stable stimulation settings may pose a greater limitation in the pediatric population.…”

Section: Neurosurgical Managementmentioning

confidence: 99%

Neurosurgical and pharmacological management of dystonia

Mohamed,

Faragalla,

Khan

et al. 2024

World J Psychiatry

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Dystonia characterizes a group of neurological movement disorders characterized by abnormal muscle movements, often with repetitive or sustained contraction resulting in abnormal posturing. Different types of dystonia present based on the affected body regions and play a prominent role in determining the potential efficacy of a given intervention. For most patients afflicted with these disorders, an exact cause is rarely identified, so treatment mainly focuses on symptomatic alleviation. Pharmacological agents, such as oral anticholinergic administration and botulinum toxin injection, play a major role in the initial treatment of patients. In more severe and/or refractory cases, focal areas for neurosurgical intervention are identified and targeted to improve quality of life. Deep brain stimulation (DBS) targets these anatomical locations to minimize dystonia symptoms. Surgical ablation procedures and peripheral denervation surgeries also offer potential treatment to patients who do not respond to DBS. These management options grant providers and patients the ability to weigh the benefits and risks for each individual patient profile. This review article explores these pharmacological and neurosurgical management modalities for dystonia, providing a comprehensive assessment of each of their benefits and shortcomings.

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Deep Brain Stimulation for Pediatric Dystonia: A Review of the Literature and Suggested Programming Algorithm

Cited by 12 publications

References 89 publications

Network localization of pediatric lesion-induced dystonia

Network localization of pediatric lesion-induced dystonia

Novel utilization of deep brain stimulation in the pedunculopontine nucleus with globus pallidus internus for treatment of childhood-onset dystonia

Neurosurgical and pharmacological management of dystonia

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