284 research outputs found
Neuroimaging phenotypes of CSF1R-related leukoencephalopathy: Systematic review, meta-analysis, and imaging recommendations
Colony-stimulating factor 1 receptor (CSF1R)-related leukoencephalopathy is a rare but fatal microgliopathy. The diagnosis is often delayed due to multifaceted symptoms that can mimic several other neurological disorders. Imaging provides diagnostic clues that help identify cases. The objective of this study was to integrate the literature on neuroimaging phenotypes of CSF1R-related leukoencephalopathy. A systematic review and meta-analysis were performed for neuroimaging findings of CSF1R-related leukoencephalopathy via PubMed, Web of Science, and Embase on 25 August 2021. The search included cases with confirmed CSF1R mutations reported under the previous terms hereditary diffuse leukoencephalopathy with spheroids, pigmentary orthochromatic leukodystrophy, and adult-onset leukoencephalopathy with axonal spheroids and pigmented glia. In 78 studies providing neuroimaging data, 195 cases were identified carrying CSF1R mutations in 14 exons and five introns. Women had a statistically significant earlier age of onset (p = 0.041, 40 vs 43 years). Mean delay between symptom onset and neuroimaging was 2.3 years. Main magnetic resonance imaging (MRI) findings were frontoparietal white matter lesions, callosal thinning, and foci of restricted diffusion. The hallmark computed tomography (CT) finding was white matter calcifications. Widespread cerebral hypometabolism and hypoperfusion were reported using positron emission tomography and single-photon emission computed tomography. In conclusion, CSF1R-related leukoencephalopathy is associated with progressive white matter lesions and brain atrophy that can resemble other neurodegenerative/-inflammatory disorders. However, long-lasting diffusion restriction and parenchymal calcifications are more specific findings that can aid the differential diagnosis. Native brain CT and brain MRI (with and without a contrast agent) are recommended with proposed protocols and pictorial examples are provided
Dilated Virchow-Robin spaces are a marker for arterial disease in multiple sclerosis
BACKGROUND
Virchow-Robin spaces (VRS) have been associated with neurodegeneration and neuroinflammation. However, it remains uncertain to what degree non-dilated or dilated VRS reflect specific features of neuroinflammatory pathology. Thus, we aimed at investigating the clinical relevance of VRS as imaging biomarker in multiple sclerosis (MS) and to correlate VRS to their histopathologic signature.
METHODS
In a cohort study comprising 142 MS patients and 30 control subjects, we assessed the association of non-dilated and dilated VRS to clinical and magnetic resonance imaging (MRI) outcomes. Findings were corroborated in a validation cohort comprising 63 MS patients. Brain blocks from 6 MS patients and 3 non-MS controls were histopathologically processed to correlate VRS to their tissue substrate.
FINDINGS
In our actively treated clinical cohort, the count of dilated centrum semiovale VRS was associated with increased T1 and T2 lesion volumes. There was no systematic spatial colocalization of dilated VRS with MS lesions. At tissue level, VRS mostly corresponded to arteries and were not associated with MS pathological hallmarks. Interestingly, in our ex vivo cohort comprising mostly progressive MS patients, dilated VRS in MS were associated with signs of small vessel disease.
INTERPRETATION
Contrary to prior beliefs, these observations suggest that VRS in MS do not associate with an accumulation of immune cells. But instead, these findings indicate vascular pathology as a driver and/or consequence of neuroinflammatory pathology for this imaging feature.
FUNDING
NIH, Swedish Society for Medical Research, Swiss National Science Foundation and University of Zurich
Short range ferromagnetism and spin glass state in
Dynamic magnetic properties of are
reported. The system appears to attain local ferromagnetic order at
K. Below this temperature the low field
magnetization becomes history dependent, i.e. the zero field cooled (ZFC) and
field cooled (FC) magnetization deviate from each other and closely logarithmic
relaxation appears at our experimental time scales (0.3- sec). The zero
field cooled magnetization has a maximum at K,
whereas the field cooled magnetization continues to increase, although less
sharply, also below this temperature. Surprisingly, the dynamics of the system
shows non-equilibrium spin glass (SG) features not only below the maximum in
the ZFC magnetization, but also in the temperature region between this maximum
and . The aging and temperature cycling experiments show only
quantitative differences in the dynamic behavior above and below the maximum in
the ZFC-magnetization; similarly, memory effects are observed in both
temperature regions. We attribute the high temperature behavior to the
existence of clusters of short range ferromagnetic order below
; the configuration evolves into a conventional spin glass
state at temperatures below .Comment: REVTeX style; 8 pages, 8 figure
Strong rejuvenation in a chiral-glass superconductor
The glassy paramagnetic Meissner phase of a BiSrCaCuO
superconductor ( = 8.18) is investigated by squid magnetometry, using
``dc-memory'' experiments employed earlier to study spin glasses. The
temperature dependence of the zero-field-cooled and thermo-remanent
magnetization is recorded on re-heating after specific cooling protocols, in
which single or multiple halts are performed at constant temperatures. The
'spin' states equilibrated during the halts are retrieved on re-heating. The
observed memory and rejuvenation effects are similar to those observed in
Heisenberg-like spin glasses.Comment: REVTeX 4 style; 5 pages, 5 figure
Anthrax outbreak in a Swedish beef cattle herd - 1st case in 27 years: Case report
After 27 years with no detected cases, an outbreak of anthrax occurred in a beef cattle herd in the south of Sweden. The outbreak was unusual as it occurred in winter, in animals not exposed to meat-and-bone meal, in a non-endemic country
Genome-wide association study and scan for signatures of selection point to candidate genes for body temperature maintenance under the cold stress in Siberian cattle populations
Design of new highly productive livestock breeds, well-adapted to local climatic conditions is one of the aims of modern agriculture and breeding. The genetics underlying economically important traits in cattle are widely studied, whereas our knowledge of the genetic mechanisms of adaptation to local environments is still scarce. To address this issue for cold climates we used an integrated approach for detecting genomic intervals related to body temperature maintenance under acute cold stress. Our approach combined genome-wide association studies (GWAS) and scans for signatures of selection applied to a cattle population (Hereford and Kazakh Whiteheaded beef breeds) bred in Siberia. We utilized the GGP HD150K DNA chip containing 139,376 single nucleotide polymorphism markers
Rapid Identification of Bio-Molecules Applied for Detection of Biosecurity Agents Using Rolling Circle Amplification
Detection and identification of pathogens in environmental samples for biosecurity applications are challenging due to the strict requirements on specificity, sensitivity and time. We have developed a concept for quick, specific and sensitive pathogen identification in environmental samples. Target identification is realized by padlock- and proximity probing, and reacted probes are amplified by RCA (rolling-circle amplification). The individual RCA products are labeled by fluorescence and enumerated by an instrument, developed for sensitive and rapid digital analysis. The concept is demonstrated by identification of simili biowarfare agents for bacteria (Escherichia coli and Pantoea agglomerans) and spores (Bacillus atrophaeus) released in field
Disgust Sensitivity and the Neurophysiology of Left- Right Political Orientations
Disgust has been described as the most primitive and central of emotions. Thus, it is not surprising that it shapes behaviors
in a variety of organisms and in a variety of contexts—including homo sapien politics. People who believe they would be
bothered by a range of hypothetical disgusting situations display an increased likelihood of displaying right-of-center rather
than left-of-center political orientations. Given its primal nature and essential value in avoiding pathogens disgust likely has
an effect even without registering in conscious beliefs. In this article, we demonstrate that individuals with marked
involuntary physiological responses to disgusting images, such as of a man eating a large mouthful of writhing worms, are
more likely to self-identify as conservative and, especially, to oppose gay marriage than are individuals with more muted
physiological responses to the same images. This relationship holds even when controlling for the degree to which
respondents believe themselves to be disgust sensitive and suggests that people’s physiological predispositions help to
shape their political orientations
Localism in Finland : The changing role and current crisis of the Finnish municipal system
Peer reviewe
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