Alfvenic waves in polar spicules

Context. For investigating spicules from the photosphere to coronal heights, the new Hinode/SOT long series of high resolution observations from Space taken in CaII H line emission offers an improved way to look at their remarkable dynamical behavior using images free of seeing effects. They should be put in the context of the huge amount of already accumulated material from ground-based instruments, including high- resolution spectra of off-limb spicules. Results. The surge-like behavior of solar polar region spicules supports the untwisting multi-component interpretation of spicules exhibiting helical dynamics. Several tall spicules are found with (i) upward and downward flows similar at lower and middle-levels, the rate of upward motion being slightly higher at high levels; (ii) the left and right-hand velocities are also increasing with height; (iii) a large number of multi-component spicules show shearing motion of both left-handed and right-handed senses occurring simultaneously, which might be understood as twisting (or untwisting) threads. The number of turns depends on the overall diameter of the structure made of components and changes from at least one turn for the smallest structure to at most two or three turns for surge-like broad structures; the curvature along the spicule corresponds to a low turn number similar to a transverse kink mode oscillation along the threads.Comment: 8 pages, 10 figures, Accepted in Astronomy and Astrophysic

The usual suspects: How psychological motives and thinking styles predict the endorsement of well-known and COVID-19 conspiracy beliefs

Research on belief in conspiracy theories identified many predictors but often failed to investigate them together. In the present study, we tested how the most important predictors of beliefs in conspiracy theories explain endorsing COVID‐19 and non‐COVID‐19 conspiracy theories and conspiracy mentality. Apart from these three measures of conspiratorial thinking, participants (N = 354) completed several measures of epistemic, existential, and social psychological motives, as well as cognitive processing variables. While many predictors had significant correlations, only three consistently explained conspiratorial beliefs when included in one model: higher spirituality (specifically eco‐awareness factor), higher narcissism, and lower analytical thinking. Compared to the other two conspiratorial measures, predictors less explained belief in COVID‐19 conspiracy theories, but this depended on items' content. We conclude that the same predictors apply to belief in both COVID and non‐COVID conspiracies and identify New Age spirituality as an important contributor to such beliefs

Biallelic SQSTM1 mutations in early-onset, variably progressive neurodegeneration.

OBJECTIVE: To characterize clinically and molecularly an early-onset, variably progressive neurodegenerative disorder characterized by a cerebellar syndrome with severe ataxia, gaze palsy, dyskinesia, dystonia, and cognitive decline affecting 11 individuals from 3 consanguineous families. METHODS: We used whole-exome sequencing (WES) (families 1 and 2) and a combined approach based on homozygosity mapping and WES (family 3). We performed in vitro studies to explore the effect of the nontruncating SQSTM1 mutation on protein function and the effect of impaired SQSTM1 function on autophagy. We analyzed the consequences of sqstm1 down-modulation on the structural integrity of the cerebellum in vivo using zebrafish as a model. RESULTS: We identified 3 homozygous inactivating variants, including a splice site substitution (c.301+2T>A) causing aberrant transcript processing and accelerated degradation of a resulting protein lacking exon 2, as well as 2 truncating changes (c.875_876insT and c.934_936delinsTGA). We show that loss of SQSTM1 causes impaired production of ubiquitin-positive protein aggregates in response to misfolded protein stress and decelerated autophagic flux. The consequences of sqstm1 down-modulation on the structural integrity of the cerebellum in zebrafish documented a variable but reproducible phenotype characterized by cerebellum anomalies ranging from depletion of axonal connections to complete atrophy. We provide a detailed clinical characterization of the disorder; the natural history is reported for 2 siblings who have been followed up for >20 years. CONCLUSIONS: This study offers an accurate clinical characterization of this recently recognized neurodegenerative disorder caused by biallelic inactivating mutations in SQSTM1 and links this phenotype to defective selective autophagy

Phenotypic continuum of NFU1-related disorders.

Bi-allelic variants in Iron-Sulfur Cluster Scaffold (NFU1) have previously been associated with multiple mitochondrial dysfunctions syndrome 1 (MMDS1) characterized by early-onset rapidly fatal leukoencephalopathy. We report 19 affected individuals from 10 independent families with ultra-rare bi-allelic NFU1 missense variants associated with a spectrum of early-onset pure to complex hereditary spastic paraplegia (HSP) phenotype with a longer survival (16/19) on one end and neurodevelopmental delay with severe hypotonia (3/19) on the other. Reversible or irreversible neurological decompensation after a febrile illness was common in the cohort, and there were invariable white matter abnormalities on neuroimaging. The study suggests that MMDS1 and HSP could be the two ends of the NFU1-related phenotypic continuum

Biallelic MED27 variants lead to variable ponto-cerebello-lental degeneration with movement disorders.

MED27 is a subunit of the Mediator multiprotein complex, which is involved in transcriptional regulation. Biallelic MED27 variants have recently been suggested to be responsible for an autosomal recessive neurodevelopmental disorder with spasticity, cataracts and cerebellar hypoplasia. We further delineate the clinical phenotype of MED27-related disease by characterizing the clinical and radiological features of 57 affected individuals from 30 unrelated families with biallelic MED27 variants. Using exome sequencing and extensive international genetic data sharing, 39 unpublished affected individuals from 18 independent families with biallelic missense variants in MED27 have been identified (29 females, mean age at last follow-up 17 ± 12.4 years, range 0.1-45). Follow-up and hitherto unreported clinical features were obtained from the published 12 families. Brain MRI scans from 34 cases were reviewed. MED27-related disease manifests as a broad phenotypic continuum ranging from developmental and epileptic-dyskinetic encephalopathy to variable neurodevelopmental disorder with movement abnormalities. It is characterized by mild to profound global developmental delay/intellectual disability (100%), bilateral cataracts (89%), infantile hypotonia (74%), microcephaly (62%), gait ataxia (63%), dystonia (61%), variably combined with epilepsy (50%), limb spasticity (51%), facial dysmorphism (38%) and death before reaching adulthood (16%). Brain MRI revealed cerebellar atrophy (100%), white matter volume loss (76.4%), pontine hypoplasia (47.2%) and basal ganglia atrophy with signal alterations (44.4%). Previously unreported 39 affected individuals had seven homozygous pathogenic missense MED27 variants, five of which were recurrent. An emerging genotype-phenotype correlation was observed. This study provides a comprehensive clinical-radiological description of MED27-related disease, establishes genotype-phenotype and clinical-radiological correlations and suggests a differential diagnosis with syndromes of cerebello-lental neurodegeneration and other subtypes of 'neuro-MEDopathies'

Knowledge, attitude, and practice of complementary and alternative medicine: a survey of Iranian nurses

Background: Development of complementary and alternative medicine (CAM) usage makes it necessary for nurses to be up to date about these treatments. However, much information is needed about the level of knowledge, attitude and practice of nurses in this field. Aims: The present study was conducted to determine the level of knowledge, attitude and practice of CAM in nurses working in Iranian hospitals. Methods: It was a descriptive study. A researcher-made questionnaire was completed by nurses working in four hospitals. Descriptive statistics were used to analyse the data. Results: Two hundred and thirty-three nurses completed the questionnaire (response rate = 83.8). Most nurses had little knowledge of CAM. The most familiar methods were Nutrition, Massage Therapy and Herbal Medicine. Of the nurses, 71.1 believed that CAM would be effective in disease treatment, showing their positive attitude toward CAM; 1.56 of nurses used CAM, and most nurses did not use CAM in clinical settings. Conclusions: Although nurses have a positive attitude toward CAM, their knowledge level is low. Regarding nurses� role in patients� treatment and the importance of their capability to treat, the need to increase nurses� knowledge of CAM is felt, and its education should be included in the nursing curriculum. © The Author(s) 2020