Aim: Studies on the use of intrathecal baclofen (ITB) for ambulant adults with spasticity and/or dystonia of cerebral origin are scarce, and are even more limited for children and adolescents. This systematic review investigates the use of ITB to improve walking, transfer ability, and gross motor activities in ambulant children and adolescents with spasticity and/or dystonia of cerebral origin.
Method: Electronic databases (MEDLINE, CINAHL, PsycINFO, EMBASE, full Cochrane Library, and PEDro) were searched from the earliest date available until March 2011 using combined subject headings and free text if supported by the databases. Studies were included if they had examined individuals who: (1) received ITB therapy by any method (bolus injection, an external delivery system, or an implanted pump); (2) had spasticity and/or dystonia of cerebral origin; (3) were able to ambulate with or without a walking device, i.e. individuals with cerebral palsy (CP) who were in levels I to III of the Gross Motor Function Classification System or individuals with similar functional mobility if they did not have CP; and (4) were aged 18 years or under. Publications in English in peer-reviewed journals reporting any type of research design, except reviews and expert opinions, were included. Studies were excluded if participants had spasticity and/or dystonia of spinal origin and if baclofen was administered only orally. Studies that compared ITB with other interventions such as botulinum toxin were also excluded.
Results: Two independent reviewers scored 16 studies against the guidelines for developing systematic reviews from the American Academy of Cerebral Palsy and Developmental Medicine (AACPDM).
Interpretation: Fifteen studies were of levels IV or V evidence and only one of level II according to the evidence levels of the AACPDM guidelines, but all were of low quality. No study was found on the use of ITB in ambulant children or adolescents with dystonia of cerebral origin. Not all studies used objective outcome measures to assess the ambulation, transfer ability, and gross motor activities of the participants. A proportion of participants showed improvement in all these areas but adverse events were common. A proportion of participants compromised their ambulatory and transfer abilities after ITB. There was no evidence to support the clinical use of ITB in ambulant individuals with hypertonicity without further rigorous longitudinal studies.
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http://dx.doi.org/10.1111/j.1469-8749.2011.03992.x | DOI Listing |
Int J Mol Sci
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Institute for Maternal and Child Health IRCCS Burlo Garofolo, Via dell'Istria, 65, 34137 Trieste, Italy.
Pathogenic variants in , encoding dynamin-like protein-1 (DRP1), cause a lethal encephalopathy. DRP1 defective function results in altered mitochondrial networks, characterized by elongated/spaghetti-like, highly interconnected mitochondria. We validated in yeast the pathogenicity of a de novo variant identified by whole exome sequencing performed more than 10 years after the patient's death.
View Article and Find Full Text PDFJ Neurosurg
January 2025
1Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing.
Objective: The aim of this study was to evaluate outcomes of deep brain stimulation (DBS) for Meige syndrome, compare the efficacy of globus pallidus internus (GPi) and subthalamic nucleus (STN) as targets, and identify potential outcome predictors.
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Brain Sci
January 2025
Unidad de Trastornos del Movimiento y Sueño, Hospital General Dr. Manuel Gea González, Calzada de Tlalpan 4800, Mexico City 14080, Mexico.
Advanced magnetic resonance imaging (MRI) techniques are transforming the study of movement disorders by providing valuable insights into disease mechanisms. This narrative review presents a comprehensive overview of their applications in this field, offering an updated perspective on their potential for early diagnosis, disease monitoring, and therapeutic evaluation. Emerging MRI modalities such as neuromelanin-sensitive imaging, diffusion-weighted imaging, magnetization transfer imaging, and relaxometry provide sensitive biomarkers that can detect early microstructural degeneration, iron deposition, and connectivity disruptions in key regions like the substantia nigra.
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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.
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PLoS Biol
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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.
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