Background: The nonmotor symptom spectrum of Parkinson's disease (PD) includes progressive cognitive decline mainly in late stages of the disease. The aim of this study was to map the patterns of altered structural connectivity of patients with PD with different cognitive profiles ranging from cognitively unimpaired to PD-associated dementia.
Methods: Diffusion tensor imaging and neuropsychological data from the observational multicentre LANDSCAPE study were analyzed. A total of 134 patients with PD with normal cognitive function (56 PD-N), mild cognitive impairment (67 PD-MCI), and dementia (11 PD-D) as well as 72 healthy controls were subjected to whole-brain-based fractional anisotropy mapping and covariance analysis with cognitive performance measures.
Results: Structural data indicated subtle changes in the corpus callosum and thalamic radiation in PD-N, whereas severe white matter impairment was observed in both PD-MCI and PD-D patients including anterior and inferior fronto-occipital, uncinate, insular cortices, superior longitudinal fasciculi, corona radiata, and the body of the corpus callosum. These regional alterations were demonstrated for PD-MCI and were more pronounced in PD-D. The pattern of involved regions was significantly correlated with the Consortium to Establish a Registry for Alzheimer's Disease (CERAD) total score.
Conclusions: The findings in PD-N suggest impaired cross-hemispherical white matter connectivity that can apparently be compensated for. More pronounced involvement of the corpus callosum as demonstrated for PD-MCI together with affection of fronto-parieto-temporal structural connectivity seems to lead to gradual disruption of cognition-related cortico-cortical networks and to be associated with the onset of overt cognitive deficits. The increase of regional white matter damage appears to be associated with the development of PD-associated dementia.
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http://dx.doi.org/10.1177/1756286419843447 | DOI Listing |
Eur J Med Genet
December 2024
CHU Lille, Institut de Génétique Médicale, F-59000 Lille, France; Univ. Lille, ULR7364 - RADEME - Maladies RAres du DEveloppement embryonnaire et du Métabolisme, F-59000 Lille, France. Electronic address:
The X-linked NONO gene encodes Non-Pou Domain-Containing Octamer-Binding Protein, a multifunctional member of the DBHS family involved in transcriptional regulation, RNA splicing and DNA repair. Pathogenic variants in NONO cause Intellectual Developmental Disorder, X-linked Syndromic (MIM #300967), characterised by intellectual disability, neurodevelopmental delay, cardiomyopathy, such as left ventricular non-compaction (LVNC), and congenital heart defects such as including atrial septal defect (ASD), ventricular septal defect (VSD), patent ductus arteriosus (PDA), and patent foramen ovale (PFO). This study reports three new patients with pathogenic hemizygous frameshift variants in NONO identified with exome sequencing, broadening the clinical presentation.
View Article and Find Full Text PDFBMC Neurol
December 2024
Department of Neurology, Peking University Shenzhen Hospital, Shenzhen, Guangdong, 518036, China.
Neuronal intranuclear inclusion disease (NIID) is a rare progressive neurodegenerative disease with a characteristic pathological feature of eosinophilic hyaluronan inclusions in the nervous system and internal organs. The identification of GGC-repeat expansions in the Notch 2 N-terminal like C (NOTCH2NLC) gene facilitates the accurate diagnosis of NIID. Due to its rareness and high clinical heterogeneity, the diagnosis of NIID is often delayed or missed.
View Article and Find Full Text PDFPak J Med Sci
December 2024
Dr. Asif Bashir, MD, FAANS, FACS Professor of Neurosurgery, Department of Neurosurgery Unit-I, Punjab Institute of Neurosciences, Lahore, Pakistan.
Objectives: To evaluate the precision and safety of a novel technique of free-hand frameless pinless AXIEM™-based navigation guided biopsy of deep-seated brain lesions in a low-middle income country.
Methods: This retrospective study included 45 patients who underwent free-hand frameless pinless AXIEM™-based navigation guided biopsy of deep-seated brain lesions using the Medtronic-Stealth S7 system over a 5-year period (January 2019 to December 2023) at the Department of Neurosurgery, Punjab Institute of Neurosciences, Lahore, Pakistan.
Results: A total of 45 patients were included in this study.
Brain Commun
December 2024
Division of Neurology, Department of Medicine, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok 10400, Thailand.
Following a unilateral post-chiasmal lesion of the geniculo-striate pathway, patients develop homonymous visual field defects. Using classical perimetry, patients with 'complete' homonymous hemianopia are unaware of stimuli in the affected hemifield. However, some show preserved vision in the affected hemifield in which the conscious perception of moving stimuli is preserved (Riddoch phenomenon).
View Article and Find Full Text PDFNeurosci Biobehav Rev
December 2024
School of Psychology, University of Leeds, Leeds LS2 9JT, UK.
The corpus callosum plays a critical role in inter-hemispheric communication by coordinating the transfer of sensory, motor, cognitive, and emotional information between the two hemispheres. However, as part of the normal aging process, the corpus callosum undergoes significant structural changes, including reductions in both its size and microstructural integrity. These age-related alterations can profoundly impact the brain's ability to coordinate functions across hemispheres, leading to a decline in various aspects of sensory processing, motor coordination, cognitive functioning, and emotional regulation.
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