Approximately 10% of dementia patients have idiopathic normal pressure hydrocephalus (iNPH), an expansion of the cerebrospinal fluid (CSF)-filled brain ventricles. iNPH and Alzheimer’s disease (AD) ...both exhibit sleep disturbances, build-up of brain metabolic wastes and amyloid-β (Aβ) plaques, perivascular reactive astrogliosis, and mislocalization of astrocyte aquaporin-4 (AQP4). The glia–lymphatic (glymphatic) system facilitates brain fluid clearance and waste removal during sleep via glia-supported perivascular channels. Human studies have implicated impaired glymphatic function in both AD and iNPH. Continued investigation into the role of glymphatic system biology in AD and iNPH models could lead to new strategies to improve brain health by restoring homeostatic brain metabolism and CSF dynamics.
iNPH constitutes ∼10% of the 50 million people currently diagnosed with a dementia-related disorder. This is expected to exceed 150 million by 2050.iNPH and AD share multiple clinical and pathologic features such as Aβ deposition, cerebrovascular inflammation, impaired localization of perivascular AQP4, and sleep disturbances.Although glymphatic system dysfunction has been extensively studied in AD, it has not yet been thoroughly examined in model systems of iNPH and other types of hydrocephalus.Several studies analyzing brain magnetic resonance imaging of human iNPH patients have shown reduced perivascular influx and efflux of intrathecally injected contrast agent compared to controls, suggesting impairment of glymphatic function iNPH.The relationship between glymphatic system function and iNPH requires further investigation because it may point toward identifiable risk factors or therapeutic targets.
Systemic effects of altered serum copper processing in Wilson Disease (WD) might induce myocardial copper deposition and consequently myocardial dysfunction and structural remodeling. This study ...sought to investigate the prevalence, manifestation and predictors of myocardial tissue abnormalities in WD patients.
We prospectively enrolled WD patients and an age-matched group of healthy individuals. We applied cardiovascular magnetic resonance (CMR) to analyze myocardial function, strain, and tissue characteristics. A subgroup analysis of WD patients with predominant neurological (WD-neuro
) or hepatic manifestation only (WD-neuro
) was performed.
Seventy-six patients (37 years (27-49), 47% women) with known WD and 76 age-matched healthy control subjects were studied. The prevalence of atrial fibrillation in WD patients was 5% and the prevalence of symptomatic heart failure was 2.6%. Compared to healthy controls, patients with WD had a reduced left ventricular global circumferential strain (LV-GCS), and also showed abnormalities consistent with global and regional myocardial fibrosis. WD-neuro
patients presented with more severe structural remodeling and functional impairment when compared to WD-neuro
patients.
In a large cohort, WD was not linked to a distinct cardiac phenotype except CMR indexes of myocardial fibrosis. More research is warranted to assess the prognostic implications of these findings.
This trial is registered at the local institutional ethics committee (S-188/2018).
PDF
