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Background: Cervical dystonia (CD) is characterized by involuntary contractions of the cervical muscles. Data on long-term effectiveness of deep brain stimulation (DBS) are rare. The aim of this study was to evaluate the longitudinal ten years treatment efficacy of DBS in the globus pallidus internus (GPI).
Methods: A retrospective single-center data analysis was performed on patients with idiopathic CD, who were treated with GPI DBS for at least 10 years. TWSTR severity score and individual sub-items were compared between pre and post DBS surgery (n = 15) over time.
Results: There was a significant and persistent positive effect regarding the severity of TWSTRS between the conditions immediately before and 1, 5, and 10 years after establishment of GPI DBS (mean difference: 6.6-7 ± 1.6). Patients with increasing CD complexity showed a poorer response to established treatment forms, such as injection of botulinum toxin and were thus DBS candidates. Especially a predominant torticollis was significantly improved by DBS.
Conclusion: GPI DBS is an effective procedure especially in severely affected patients with a positive 10-year outcome. It should be considered in more complex CD-forms or predominant torticollis.
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http://dx.doi.org/10.1186/s42466-022-00214-8 | DOI Listing |
Pediatr Neurol
August 2025
Movement Disorders Unit, Pediatric Neurology Department, Institut de Recerca, Hospital Sant Joan de Déu Barcelona, Barcelona, Spain; European Reference Network for Rare Neurological Diseases (ERN-RND), Barcelona, Spain; Biomedical Network Research Centre on Rare Diseases (CIBERER), Instituto de Sal
Background: Biallelic pathogenic variants in the HPCA gene cause HPCA-associated dystonia (DYT-HPCA), a rare autosomal recessive disorder characterized by generalized dystonia and complex motor symptoms. HPCA encodes hippocalcin, a Ca sensor that modulates neuronal activity through K channel activation. Here, we describe the clinical and molecular features of two children with novel HPCA variants and assess the impact of deep brain stimulation (DBS) (globus pallidus internus [Gpi]-DBS) on their movement disorders.
View Article and Find Full Text PDFMov Disord Clin Pract
August 2025
Neurology Unit, ASST Santi Paolo e Carlo, San Paolo University Hospital Milan, Milan, Italy.
Int J Mol Sci
August 2025
Department of Neurology, Faculty of Health Sciences, Medical University of Warsaw, Ul. Zwirki i Wigury 61, 02-091 Warsaw, Poland.
Dystonia-myoclonus syndrome is a rare neurological condition characterized by involuntary muscle contractions and myoclonic jerks, significantly impairing daily functioning. Pharmacological management is often ineffective, prompting consideration of alternative therapeutic interventions such as deep brain stimulation (DBS). This report describes a novel clinical case involving a 38-year-old female with severe dystonic and myoclonic symptoms associated with a pathogenic mutation in the KCNN2 gene (DYT34).
View Article and Find Full Text PDFBackground: Deep brain stimulation of the globus pallidus internus (DBS-GPi) has shown efficacy in adult patients with SGCE-related myoclonus dystonia. However, evidence regarding its impact in pediatric populations is limited.
Objectives: The aim was to evaluate motor and non-motor outcomes following DBS-GPi intervention in children and adolescents with SGCE-MD.
AJNR Am J Neuroradiol
August 2025
From the Department of Radiology, Chinese PLA General Hospital/Medical School of Chinese PLA, Beijing, China (H.L., Y.X., J.H., X.W., X.W., J.H., Z.L., X.L.), and Department of Neurosurgery, Chinese PLA General Hospital, Beijing, China (L.P.).
Background And Purpose: Magnetic resonance-guided focused ultrasound (MRgFUS) is a promising and noninvasive treatment for medication-refractory essential tremor (ET). However, the mechanism of long-term postoperative brain structural remodeling remains unclear. This study aimed to investigate the changes in gray matter volume (GMV) before and after MRgFUS, as well as to explore the relationship between GMV changes and tremor control.
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