Background: Behcet's disease (BD) may present with life threating complications including neurological and cardiovascular involvement. Neuro-Behcet's disease (NBD) is one of the most important causes of morbidity and mortality in patients with BD. The aim of the present study is to investigate whether patients with NBD are different than BD patients with other manifestations in terms of subclinical myocardial dysfunction.
Methods: Forty patients with NBD (23 female, mean age: 42.4 ± 9.4 years), 40 patients with BD (9 female, mean age: 39.7 ± 9.0 years) and 40 controls (20 male, mean age: 41.8 ± 6.5 years) were consecutively included in the study. All subjects underwent a transthoracic echocardiography for evaluation of left ventricular (LV) and atrial (LA) functions with two-dimensional (2D) speckle tracking echocardiography (STE).
Results: Baseline characteristics, clinical data, LV dimensions, systolic and diastolic functions were all in normal range among the groups. LV global longitudinal strain (LV-GLS) was significantly lower in patients with NBD and BD patients without neurologic involvement compared to controls. LA conduit strain was significantly lower in patients with NBD compared to controls. Patients with both parenchymal NBD and vascular NBD manifestations had significantly lower LV-GLS and LA conduit strain compared to controls. Linear regression analysis demonstrated that among cardiovascular risk factors only presence of NBD was the independent predictor of LV-GLS.
Conclusions: BD is associated with impaired LV and LA functions. LV-GLS and LA conduit strains of the patients with NBD were lower. NBD was an independent predictor of LV-GLS, suggesting a link between neurological manifestations and cardiac dysfunction in BD patients.
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http://dx.doi.org/10.1080/00015385.2021.1965354 | DOI Listing |
Neurobiol Dis
January 2025
The University of Texas Southwestern Medical Center, Department of Neurology, Dallas, TX, United States of America; The University of Texas Southwestern Medical Center, Department of Psychiatry, Dallas, TX, United States of America; The University of Texas Southwestern Medical Center, Department of Pediatrics, Dallas, TX, United States of America; The University of Texas Southwestern Medical Center, Department of Neuroscience, Dallas, TX, United States of America. Electronic address:
Loss of function in the subunits of the GTPase-activating protein (GAP) activity toward Rags-1 (GATOR1) complex, an amino-acid sensitive negative regulator of the mechanistic target of rapamycin complex 1 (mTORC1), is implicated in both genetic familial epilepsies and NDDs (Baldassari et al., 2018). Previous studies have found seizure phenotypes and increased activity resulting from conditional deletion of GATOR1 function from forebrain excitatory neurons (Yuskaitis et al.
View Article and Find Full Text PDFClin Ophthalmol
December 2024
Department of Sense Organs, Sapienza University, Rome, Italy.
Purpose: Osteogenesis imperfecta (OI) is a rare hereditary disorder of the connective tissue. Despite recent attention to corneal abnormalities in OI, understanding remains limited. This study aimed to comprehensively evaluate corneal changes in a large sample of OI patients compared to controls using in vivo confocal microscopy (IVCM).
View Article and Find Full Text PDFNeurobiol Dis
December 2024
Department of Physiology & Neuroscience, Zilkha Neurogenetic Institute, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA. Electronic address:
Huntington's disease (HD) is caused by the expansion of a CAG repeat, encoding a string of glutamines (polyQ) in the first exon of the huntingtin gene (HTTex1). This mutant huntingtin protein (mHTT) with extended polyQ forms aggregates in cortical and striatal neurons, causing cell damage and death. The retina is part of the central nervous system (CNS), and visual deficits and structural abnormalities in the retina of HD patients have been observed.
View Article and Find Full Text PDFNeurobiol Dis
December 2024
The Florey Institute of Neuroscience and Mental Health, Parkville, Victoria 3052, Australia. Electronic address:
Background: Developmental and epileptic encephalopathies (DEE) are rare but severe neurodevelopmental disorders characterised by early-onset seizures often combined with developmental delay, behavioural and cognitive deficits. Treatment for DEEs is currently limited to seizure control and provides no benefits to the patients' developmental and cognitive outcomes. Genetic variants are the most common cause of DEE with KCNQ2 being one of the most frequently identified disease-causing genes.
View Article and Find Full Text PDFNeurobiol Dis
December 2024
Graduate Institute of Brain and Mind Sciences, National Taiwan University College of Medicine, Taipei 10051, Taiwan. Electronic address:
Evidence indicates that neurodegenerative diseases spread through distinct brain networks. For Parkinson's disease (PD), somatosensory abnormalities may accompany motor dysfunction in early disease stages when dopaminergic degeneration is limited to the basal ganglia. It remains unclear whether, based on the network-spread account, these abnormalities emanated from aberrant functional connectivity with the basal ganglia, and whether interventions normalizing this connectivity could reverse these abnormalities.
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