Background: GPi (Internal globus pallidus) DBS (deep brain stimulation) is recognized as a safe, reliable, reversible and adjustable treatment in patients with medically refractory dystonia.
Objectives: This report describes the long-term clinical outcome of 36 patients implanted with GPi DBS at the Neurosurgery Department of Seoul National University Hospital.
Methods: Nine patients with a known genetic cause, 12 patients with acquired dystonia, and 15 patients with isolated dystonia without a known genetic cause were included. When categorized by phenomenology, 29 patients had generalized, 5 patients had segmental, and 2 patients had multifocal dystonia. Patients were assessed preoperatively and at defined follow-up examinations postoperatively, using the Burke-Fahn-Marsden dystonia rating scale (BFMDRS) for movement and functional disability assessment. The mean follow-up duration was 47 months (range, 12-84).
Results: The mean movement scores significantly decreased from 44.88 points preoperatively to 26.45 points at 60-month follow up (N = 19, P = 0.006). The mean disability score was also decreased over time, from 11.54 points preoperatively to 8.26 points at 60-month follow up, despite no statistical significance (N = 19, P = 0.073). When analyzed the movement and disability improvement rates at 12-month follow up point, no significant difference was noted according to etiology, disease duration, age at surgery, age of onset, and phenomenology. However, the patients with DYT-1 dystonia and isolated dystonia without a known genetic cause showed marked improvement.
Conclusions: GPi DBS is a safe and efficient therapeutic method for treatment of dystonia patients to improve both movement and disability. However, this study has some limitations caused by the retrospective design with small sample size in a single-center.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4706355 | PMC |
http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0146644 | PLOS |
Parkinsonism Relat Disord
January 2025
Institute of Systems Motor Science, University Medical Center Schleswig-Holstein, University of Lübeck, Lübeck, Germany; Center for Brain, Behavior, and Metabolism, University of Lübeck, Lübeck, Germany; Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, United States; Center of Rare Diseases, University Medical Center Schleswig-Holstein, Lübeck, Germany. Electronic address:
Background: Functional neurological movement disorders are common and disabling. Little is known about their coexistence with other non-functional movement disorders and their impact on the general disease burden.
Objectives: Investigating frequency and characteristics of functional movement disorders in GCH1-positive dopa-responsive dystonia patients.
Parkinsonism Relat Disord
January 2025
Human Sensorimotor Control Laboratory, School of Kinesiology, University of Minnesota, USA; Center for Clinical Movement Science, University of Minnesota, USA.
Introduction: Cervical dystonia (CD) is characterized by involuntary neck muscle spasms that lead to abnormal head movements or postures. It is associated with somatosensory (tactile and proprioceptive) dysfunction. Here we tested whether vibro-tactile stimulation (VTS) of the cervical muscles constitutes a non-invasive form of neuromodulation of the somatosensory system that can provide temporary symptom relief for people with CD.
View Article and Find Full Text PDFPLoS Biol
January 2025
Carney Institute for Brain Science, Department of Cognitive & Psychological Sciences, Brown University, Providence, Rhode Island, United States of America.
The basal ganglia (BG) play a key role in decision-making, preventing impulsive actions in some contexts while facilitating fast adaptations in others. The specific contributions of different BG structures to this nuanced behavior remain unclear, particularly under varying situations of noisy and conflicting information that necessitate ongoing adjustments in the balance between speed and accuracy. Theoretical accounts suggest that dynamic regulation of the amount of evidence required to commit to a decision (a dynamic "decision boundary") may be necessary to meet these competing demands.
View Article and Find Full Text PDFStereotact Funct Neurosurg
January 2025
Introduction: Functional thalamic surgery is known for alleviating isolated focal hand dystonia; however, the optimal target site in the thalamus is not determined. This study aimed to identify effective sites for thalamic deep brain stimulation (DBS) in treating this condition.
Methods: Four patients presenting with focal hand dystonia underwent thalamic DBS.
Front Pediatr
January 2025
Department of Urology, The Affiliated Children's Hospital of Xiangya School of Medicine, Central South University (Hunan Children's Hospital), Changsha, China.
Objective: This study aimed to systematically evaluate the safety of cyclosporine (CsA) and tacrolimus (TAC) in pediatric nephrotic syndrome (NS) patients using real-world data from the FDA Adverse Event Reporting System (FAERS).
Methods: We analyzed adverse event (AE) reports from the FAERS database between Q4 2003 and Q2 2024, focusing on AEs associated with CsA and TAC in NS patients aged 18 years and younger. We employed three signal detection methods-Proportional Reporting Ratio (PRR), Relative Reporting Ratio (RRR), and Reporting Odds Ratio (ROR)-to assess the risk of drug-related AEs.
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