We recognize the need for longitudinal measures and mechanistic understanding of the basis for phenotype to single-neuron correlations. We clarify that pallidal neuron differs across dystonia subtypes. We highlight animal studies showing how cerebellar activity can influence pallidal neuron behavior, supporting the idea of cerebellar activity transmission to the pallidum.
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http://dx.doi.org/10.1016/j.parkreldis.2025.107350 | DOI Listing |
J Clin Neurol
March 2025
Department of Geriatric Neurology, Xiangya Hospital, Central South University, Changsha, Hunan, China.
Background And Purpose: Essential tremor with a midline distribution (Mid-ET) may represent a distinct subtype of essential tremor (ET) that primarily affects midline structures, often indicating advanced disease stage and increased severity. Recent studies have highlighted the complexity of Mid-ET, but research on neurological soft signs (NSS) in Mid-ET remains insufficient.
Methods: The patients with ET included in this cross-sectional study were divided into two subgroups based on whether or not the ET had a midline distribution: Mid-ET and No-Mid-ET.
Parkinsonism Relat Disord
February 2025
N.N. Semenov Federal Research Center for Chemical Physics, Russian Academy of Sciences, Moscow, Russia; N. N. Burdenko National Medical Research Center of Neurosurgery, Moscow, Russia; Moscow Institute of Physics and Technology, Dolgoprudny, Moscow Region, Russia.
We recognize the need for longitudinal measures and mechanistic understanding of the basis for phenotype to single-neuron correlations. We clarify that pallidal neuron differs across dystonia subtypes. We highlight animal studies showing how cerebellar activity can influence pallidal neuron behavior, supporting the idea of cerebellar activity transmission to the pallidum.
View Article and Find Full Text PDFInt J Mol Sci
February 2025
Centro Andaluz de Biología del Desarrollo (CABD-CSIC-Universidad Pablo de Olavide), 41013 Sevilla, Spain.
Neurodegeneration with brain iron accumulation (NBIA) involves a group of rare neurogenetic disorders often linked with iron overload in the basal nuclei of the brain presenting with spasticity, dystonia, muscle rigidity, neuropsychiatric symptoms, and retinal degeneration. Among NBIA subtypes, beta-propeller-protein-associated neurodegeneration (BPAN) is associated with mutations in the autophagy gene WDR45 (WD repeat domain 45). Previously, we demonstrated that WDR45 mutations in BPAN cellular models impaired autophagy, iron metabolism, and cell bioenergetics.
View Article and Find Full Text PDFDis Model Mech
February 2025
Divisions of Developmental Biology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45229, USA.
Gsx2 is a homeodomain transcription factor critical for development of the ventral telencephalon and hindbrain in mouse. Loss of Gsx2 function results in severe basal ganglia dysgenesis and defects in the nucleus tractus solitarius (nTS) of the hindbrain, together with respiratory failure at birth. De Mori et al.
View Article and Find Full Text PDFCrit Care
January 2025
Department of Neuro-Intensive Care Unit, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, China.
Background And Objectives: Antibody-negative autoimmune encephalitis (AE) is a form of encephalitis characterized by the absence of detectable autoimmune antibodies, despite immunological evidence. However, data on management of patients with antibody-negative AE in the intensive care unit (ICU) are limited. This study aimed to explore the characteristics and subtypes of antibody-negative AE, assess the effects of immunotherapy, and identify factors independently associated with poor functional outcomes in patients requiring intensive care.
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