Degeneration and Plasticity of the Optic Pathway in Alström Syndrome.

BACKGROUND AND PURPOSE: Alstrom syndrome is a rare inherited ciliopathy in which early progressive cone-rod dystrophy leads to childhood blindness. We investigated functional and structural changes of the optic pathway in Alstrom syndrome by using MR imaging to provide insight into the underlying pathogenic mechanisms. MATERIALS AND METHODS: Eleven patients with genetically proved Alstrom syndrome (mean age, 23 years; range, 6–45 years; 5 females) and 19 age- and sex-matched controls underwent brain MR imaging. The study protocol included conventional sequences, resting-state functional MR imaging, and diffusion tensor imaging. RESULTS: In patients with Alstrom syndrome, the evaluation of the occipital regions showed the following: 1) diffuse white matter volume decrease while gray matter volume decrease spared the occipital poles (voxel-based morphometry), 2) diffuse fractional anisotropy decrease and radial diffusivity increase while mean and axial diffusivities were normal (tract-based spatial statistics), and 3) reduced connectivity in the medial visual network strikingly sparing the occipital poles (independent component analysis). After we placed seeds in both occipital poles, the seed-based analysis revealed significantly increased connectivity in patients with Alstrom syndrome toward the left frontal operculum, inferior and middle frontal gyri, and the medial portion of both thalami (left seed) and toward the anterior portion of the left insula (right and left seeds). CONCLUSIONS: The protean occipital brain changes in patients with Alstrom syndrome likely reflect the coexistence of diffuse primary myelin derangement, anterograde trans-synaptic degeneration, and complex cortical reorganization affecting the anterior and posterior visual cortex to different degrees

Apparent diffusion coefficient restriction in the white matter: going beyond acute brain territorial ischemia

BACKGROUND: Reduction of apparent diffusion coefficient (ADC) values in white matter is not always ischaemic in nature. METHODS: We retrospectively analysed our MRI records featuring reduced ADC values in the centrum semiovale without grey matter involvement or significant vasogenic oedema. RESULTS: Several conditions showed the aforementioned MR findings: moose-horn lesions on coronal images in X-linked Charcot-Marie-Tooth disease; small fronto-parietal lesions in Menkes disease; marked signal abnormalities in the myelinised regions in the acute neonatal form of maple syrup urine disease; strip-like involvement of the corpus callosum in glutaric aciduria type 1; persistent periventricular parieto-occipital abnormalities in phenylketonuria; diffuse signal abnormalities with necrotic evolution in global cerebral anoxia or after heroin vapour inhalation; almost completely reversible symmetric fronto-parietal lesions in methotrexate neurotoxicity; chain-like lesions in watershed ischaemia; splenium involvement that normalises in reversible splenial lesions or leads to gliosis in diffuse axonal injury. CONCLUSION: Neuroradiologists must be familiar with these features, thereby preventing misdiagnosis and inappropriate management

A standard protocol for documenting modern and fossil ichnological data

The collection and dissemination of vertebrate ichnological data is struggling to keep up with techniques that are becoming common place in the wider palaeontological field. A standard protocol is required in order to ensure that data is recorded, presented, and archived in a manner that will be useful both to contemporary researchers, and to future generations. Primarily, our aim is to make the 3D capture of ichnological data standard practice, and to provide guidance on how such 3D data can be communicated effectively (both via the literature and other means), and archived openly and in perpetuity. We recommend capture of 3D data, and the presentation of said data in the form of photographs, false-colour images, and interpretive drawings. Raw data (3D models of traces) should always be provided in a form usable by other researchers, i.e. in an open format. If adopted by the field as a whole, the result will be a more robust and uniform literature, supplemented by unparalleled availability of datasets for future workers

Nurses' perceptions of aids and obstacles to the provision of optimal end of life care in ICU

Contains fulltext : 172380.pdf (publisher's version ) (Open Access

Tetrapod ichnology in Italy. The state of the art. Guest editorial

This year, 2020, marks the 150thanniversary of the seminal work by Giulio Curioni (1870), representing the first published scientific contribution on tetrapod footprints from Italy. We took this opportunity to discuss the current state of the art on tetrapod ichnology in our country, with a jubilee issue, titled "Tetrapod ichnology in Italy: the state of the art". The issue involves the scholars who first pioneered this discipline in Italy in the seventies of the last century, along with all the authors who have worked on the topic in recent decades, and younger generations who have just started to enthusiastically contribute to vertebrate ichnology. After briefly introducing the idea at the base of the Special Issue, as well as some aspects of the discipline and the current methodologies involved in ichnological studies, we present each of the contributions to serve the Italian ichnological heritage

Control of Glucose, Blood Pressure, and Cholesterol among Adults with Diabetes: The Brazilian National Health Survey

ABC (glucose, blood pressure and LDL-cholesterol) goals are basic standards of diabetes care. We aimed to assess ABC control and related factors in a representative sample of Brazilian adults with diabetes. We analyzed 465 adults with known diabetes in the Brazilian National Health Survey. The targets used were <7% for glycated hemoglobin (A1C); <140/90 mmHg for blood pressure; and <100 mg/dL for LDL-C, with stricter targets for the latter two for those with high cardiovascular (CVD) risk. Individual goals were attained by 46% (95% CI, 40.3–51.6%) for A1C, 51.4% (95% CI, 45.7–57.1%) for blood pressure, and 40% (95% CI, 34.5–45.6%) for LDL-C. The achievement of all three goals was attained by 12.5% (95% CI, 8.9–16.2%). Those with high CVD risk attained blood pressure and LDL-C goals less frequently. A1C control improved with increasing age and worsened with greater duration of diabetes. Achievement of at least two ABC goals decreased with increasing BMI and greater duration of diabetes. In sum, about half of those with known diabetes achieved each ABC goal and only a small fraction achieved all three goals. Better access and adherence to treatment and strategies to personalize goals according to specific priorities are of the essence

A review of the concepts of ‘axony’ and their bearing on tetrapod ichnology

Two meanings of the term axony are found in the ichnological literature. Multiple meanings may prove to be a double-edge sword, complicating scientific communication. In vertebrate ichnology the first meaning of axony relies on aspects of locomotion related to the body weight support and propulsive thrust. A second one concerns axony as a purely geometric and dimensional descriptor. These approaches are based on a static view of the impression process, implying the loss of much important information. Here we report an analysis of shallowly impressed footprints referred to the ichnotaxa Ichniotherium sphaerodactylum and Dimetropus osageorum. The analysis was carried out by considering the track registration as a dynamic process and attempting to identify and describe axony conditions during movements. Variations in the axony conditions can be understood in the light of the producer’s foot anatomy and the reciprocal relations between foot bone elements. The concept of axony can be a useful tool in ichnological practice only when it is related to the complex dynamic of locomotion and the resulting track registration. It can help in restoring the interconnections between track and trackmaker, re-establishing the biological significance of tetrapod footprints