Background: Degeneration of dopaminergic neurons within the brainstem substantia nigra (SN) is both a pathological hallmark of Parkinson's disease (PD) and a major contributor to symptom expression. Therefore, non-invasive evaluation of the SN is critical for diagnosis and evaluation of disease progression. Hyperechogenicity (HE+) on midbrain transcranial sonography (TCS) supports the clinically established diagnosis of PD. Further, postmortem studies suggest involvement of neuromelanin (NM) loss and iron deposition in nigral neurodegeneration and HE+ emergence. However, the associations between HE+ and signs of nigral NM loss and iron deposition revealed by magnetic resonance imaging (MRI) have not been examined.
Objective: To elucidate the magnetic resonance- (MR-) morphological representation of the HE+ by NM-weighted (NMI) and susceptibility-weighted MRI (SWI).
Methods: Thirty-four PD patients and 29 healthy controls (HCs) received TCS followed by NMI and SWI. From MR images, two independent raters manually identified the SN, placed seeds in non-SN midbrain areas, and performed semi-automated SN segmentation with different thresholds based on seed mean values and standard deviations. Masks of the SN were then used to extract mean area, mean signal intensity, maximal signal area, maximum signal (for NMI), and minimum signal (for SWI).
Results: There were no significant differences in NMI- and SWI-based parameters between patients and HCs, and no significant associations between HE+ extent and NMI- or SWI-based parameters.
Conclusion: HE+ on TCS appears unrelated to PD pathology revealed by NMI and SWI. Thus, TCS and MRI parameters should be considered complementary, and the pathophysiological correlates of the HE+ require further study.
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http://dx.doi.org/10.3233/JPD-213000 | DOI Listing |
Mov Disord
January 2025
Department of Neuroscience, Mayo Clinic, Jacksonville, Florida, USA.
Background: Perry syndrome (PS) is a rare and fatal hereditary autosomal dominant neurodegenerative disorder caused by mutations in dynactin (DCTN1). PS brains accumulate inclusions positive for ubiquitin, transactive-response DNA-binding protein of 43 kDa (TDP-43), and to a lesser extent dynactin.
Objectives: Little is known regarding the contributions of TDP-43, an RNA binding protein that represses cryptic exon inclusion, in PS.
Neuron
January 2025
Department of Pharmacology and Department of Pharmacy of the Second Affiliated Hospital, Key Laboratory of Medical Neurobiology of the Ministry of Health of China, School of Basic Medical Sciences, Zhejiang University School of Medicine, Hangzhou 310058, China. Electronic address:
Attention deficit hyperactivity disorder (ADHD), affecting 4% of the population, is characterized by inattention, hyperactivity, and impulsivity; however, its neurophysiological mechanisms remain unclear. Here, we discovered that deficiency of histamine H receptor (HR) in parvalbumin-positive neurons in substantia nigra pars recticulata (PV) attenuates PV neuronal activity and induces hyperactivity, impulsivity, and inattention in mice. Moreover, decreased HR expression was observed in PV in patients with ADHD symptoms and dopamine-transporter-deficient mice, whose behavioral phenotypes were alleviated by HR agonist treatment.
View Article and Find Full Text PDFJ Neurol Sci
January 2025
UniSA Clinical & Health Sciences and Alliance for Research in Exercise, Nutrition and Activity (ARENA), City East Campus, University of South Australia, GPO Box 2471, Adelaide, South Australia 5001, Australia. Electronic address:
The appearance of the substantia nigra (SN) can aid diagnosis of Parkinson's disease (PD). The effect of age and sex on the appearance of nigrosome-1 (SN subregion) on magnetic resonance imaging (MRI), and the relationship between nigrosome-1 (viewed with MRI) and SN echogenicity (viewed with transcranial ultrasound) is unknown. The study aimed to address these knowledge gaps.
View Article and Find Full Text PDFNeuroimage Clin
December 2024
Coimbra Institute for Biomedical Imaging and Translational Research (CIBIT), University of Coimbra, 3000-548 Coimbra, Portugal; Institute for Nuclear Sciences Applied to Health (ICNAS), University of Coimbra, 3000-548 Coimbra, Portugal; Faculty of Medicine, Institute of Physiology, University of Coimbra, 3004-531 Coimbra, Portugal. Electronic address:
Dysfunctional response inhibition, mediated by the striatum and its connections, is thought to underly the clinical manifestations of obsessive-compulsive disorder (OCD). However, the exact neural mechanisms remain controversial. In this study, we undertook a novel approach by positing that a) inhibition is a dynamic construct inherently susceptible to numerous failures, which require error-processing, and b) the actor-critic framework of reinforcement learning can integrate neural patterns of inhibition and error-processing in OCD with their behavioural correlates.
View Article and Find Full Text PDFComput Med Imaging Graph
January 2025
Université Clermont Auvergne, Clermont Auvergne INP, CNRS, Institut Pascal, F-63000 Clermont-Ferrand, France; Université Clermont Auvergne, CNRS, CHU Clermont-Ferrand, Clermont Auvergne INP, Institut Pascal, F-63000 Clermont-Ferrand, France.
Methods for the automated segmentation of brain structures are a major subject of medical research. The small structures of the deep brain have received scant attention, notably for lack of manual delineations by medical experts. In this study, we assessed an automated segmentation of a novel clinical dataset containing White Matter Attenuated Inversion-Recovery (WAIR) MRI images and five manually segmented structures (substantia nigra (SN), subthalamic nucleus (STN), red nucleus (RN), mammillary body (MB) and mammillothalamic fascicle (MT-fa)) in 53 patients with severe Parkinson's disease.
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