Right temporal variant frontotemporal dementia is pathologically heterogeneous: a case-series and a systematic review

Although the right temporal variant frontotemporal dementia (rtvFTD) is characterised by distinct clinical and radiological features, its underlying histopathology remains elusive. Being considered a right-sided variant of semantic variant primary progressive aphasia (svPPA), TDP-43 type C pathology has been linked to the syndrome, but this has not been studied in detail in large cohorts. In this case report and systematic review, we report the autopsy results of five subjects diagnosed with rtvFTD from our cohort and 44 single rtvFTD subjects from the literature. Macroscopic pathological evaluation of the combined results revealed that rtvFTD demonstrated either a frontotemporal or temporal evolution, even if the degeneration started in the right temporal lobe initially. FTLD-TDP type C was the most common underlying pathology in rtvFTD, however, in 64% of rtvFTD, other underlying pathologies than FTLD-TDP type C were present, such as Tau-MAPT and FTLD-TDP type A and B. Additionally, accompanying motor neuron or corticospinal tract degeneration was observed in 28% of rtvFTD patients. Our results show that in contrast to the general assumption, rtvFTD might not be a pure FTLD-TDP type C disorder, unlike its left temporal counterpart svPPA. Large sample size pathological studies are warranted to understand the diverse pathologies of the right and left temporal variants of frontotemporal dementia

Lung cryobiopsy for the diagnosis of interstitial lung diseases: A series contribution to a debated procedure

Introduction: Transbronchial cryobiopsy is an alternative to surgical biopsy for the diagnosis of fibrosing interstitial lung diseases, although the role of this relatively new method is rather controversial. Aim of this study is to evaluate the diagnostic performance and the safety of transbronchial cryobiopsy in patients with fibrosing interstitial lung disease. Materials and methods: The population in this study included patients with interstitial lung diseases who underwent cryobiopsy from May 2015 to May 2018 at the Division of Pneumology of San Giuseppe Hospital in Milan and who were retrospectively studied. All cryobiopsy procedures were performed under fluoroscopic guidance using a flexible video bronchoscope and an endobronchial blocking system in the operating room with patients under general anaesthesia. The diagnostic performance and safety of the procedure were assessed. The main complications evaluated were endobronchial bleeding and pneumothorax. All cases were studied with a multidisciplinary approach, before and after cryobiopsy. Results: Seventy-three patients were admitted to this study. A specific diagnosis was reached in 64 cases, with a diagnostic sensitivity of 88%; 5 cases (7%) were considered inadequate, 4 cases (5%) were found to be non-diagnostic. Only one major bleeding event occurred (1.4%), while 14 patients (19%) experienced mild/moderate bleeding events while undergoing bronchoscopy; 8 cases of pneumothorax (10.9%) were reported, of which 2 (2.7%) required surgical drainage. Conclusions: When performed under safe conditions and in an experienced center, cryobiopsy is a procedure with limited complications having a high diagnostic yield in fibrotic interstitial lung disease

Differentiating Vogt-Koyanagi-Harada syndrome from recurrent optic neuritis: a case report and review of the literature concerning Hispanic patients

Abstract Background First recognized at the beginning of twentieth century and named after three authors who independently described some affected patients, Vogt-Koyanagi-Harada syndrome is a rare multisystemic autoimmune disease targeting melanin-containing tissues of the eye, meninges, inner ear and skin. It predominantly affects Asian people, but also people with darker skin pigmentation such as Native Americans and Hispanics (Mestizos), whose ancestors moved from Asia across the Bering strait to North America and further down to Central and South America. Heterogenous presentation is observed, especially among different ethnic groups. Here we describe the case of an Hispanic South American patient presenting with multiple visual relapses and thus mimicking recurrent optic neuritis; we provide insights into the differential diagnosis and a brief review of the literature concerning the epidemiology of Vogt-Koyanagi-Harada syndrome in Hispanic patients compared with other ethnic groups. Case presentation A 34-year-old Ecuadorian woman presented over years with multiple relapses involving the visual system. She was investigated in both neurologic and ophthalmic clinical settings. Brain Magnetic Resonance Imaging, cerebrospinal fluid examination, Spectral Domain Optical Coherence Tomography and Fluorescein Angiography were performed. She was misdiagnosed first as an optic neuritis pointing to a demyelinating disorder, then as a posterior scleritis. Due to the protean manifestations of Vogt-Koyanagi-Harada syndrome and the incomplete clinical presentation at the beginning, the right diagnosis was made only at a later disease stage using retrospective criteria. Conclusions Hispanic patients often present without extraocular symptoms in early phases of the disease and they have globally lower rates of intertegumentary signs compared to Asian patients. The diagnosis of a multisystemic disease such as Vogt-Koyanagi-Harada syndrome is a challenge involving specialists operating in different medical fields; especially in urban multiethnic populations, rare etiologies of common symptoms have to be taken into account when performing a differential diagnosis

Suppression of the spin waves nonreciprocity due to interfacial Dzyaloshinskii Moriya interaction by lateral confinement in magnetic nanostructures

Despite the huge recent interest towards chiral magnetism related to the interfacial Dzyaloshinskii Moriya interaction (iDMI) in layered systems, there is a lack of experimental data on the effect of iDMI on the spin waves eigenmodes of laterally confined nanostructures. Here we exploit Brillouin Light Scattering (BLS) to analyze the spin wave eigenmodes of non-interacting circular and elliptical dots, as well as of long stripes, patterned starting from a Pt(3.4 nm)/CoFeB(0.8 nm) bilayer, with lateral dimensions ranging from 100 nm to 400 nm. Our experimental results, corroborated by micromagnetic simulations based on the GPU-accelerated MuMax3 software package, provide evidence for a strong suppression of the frequency asymmetry between counter-propagating spin waves (corresponding to either Stokes or anti-Stokes peaks in BLS spectra), when the lateral confinement is reduced from 400 nm to 100 nm, i.e. when it becomes lower than the light wavelength. Such an evolution reflects the modification of the spin wave character from propagating to stationary and indicates that the BLS based method of quantifying the i-DMI strength from the frequency difference of counter propagating spin waves is not applicable in the case of magnetic elements with lateral dimension below about 400 nm.Comment: Accepted for pubblication by: Physical Review

Round robin comparison on quantitative nanometer scale magnetic field measurements by magnetic force microscopy

Magnetic force microscopy (MFM) can be considered as a standard tool for nano-scale investigation of magnetic domain structures by probing the local stray magnetic field landscape of the measured sample. However, this generally provides only qualitative data. To quantify the stray magnetic fields, the MFM system must be calibrated. To that end, a transfer function (TF) approach was proposed, that, unlike point probe models, fully considers the finite extent of the MFM tip. However, albeit being comprehensive, the TF approach is not yet well established, mainly due to the ambiguities concerning the input parameters and the measurement procedure. Additionally, the calibration process represents an ill-posed problem which requires a regularization that introduces further parameters. In this paper we propose a guideline for quantitative stray field measurements by standard MFM tools in ambient conditions. All steps of the measurement and calibration procedure are detailed, including reference sample and sample under test (SUT) measurements and the data analysis. The suitability of the reference sample used in the present work for calibrated measurements on a sub-micron scale is discussed. A specific regularization approach based on a Pseudo-Wiener Filter is applied and combined with criteria for the numerical determination of a unique regularization parameter. To demonstrate the robustness of such a defined approach, a round robin comparison of magnetic field measurements was conducted by four laboratories. The guideline, the reference sample and the results of the round robin are discussed

Social cognition deficits and biometric signatures in the behavioural variant of Alzheimer’s disease

The behavioural variant of Alzheimer’s disease (bvAD) is characterized by early predominant behavioural changes, mimicking the behavioural variant of frontotemporal dementia (bvFTD), which is characterized by social cognition deficits and altered biometric responses to socioemotional cues. These functions remain understudied in bvAD. We investigated multiple social cognition components (i.e. emotion recognition, empathy, social norms and moral reasoning), using the Ekman 60 faces test, Interpersonal Reactivity Index, empathy eliciting videos, Social Norms Questionnaire and moral dilemmas, while measuring eye movements and galvanic skin response. We compared 12 patients with bvAD with patients with bvFTD (n = 14), typical Alzheimer’s disease (tAD, n = 13) and individuals with subjective cognitive decline (SCD, n = 13), using ANCOVAs and age- and sex-adjusted post hoc testing. Patients with bvAD (40.1 ± 8.6) showed lower scores on the Ekman 60 faces test compared to individuals with SCD (49.7 ± 5.0, P &lt; 0.001), and patients with tAD (46.2 ± 5.3, P = 0.05) and higher scores compared to patients with bvFTD (32.4 ± 7.3, P = 0.002). Eye-tracking during the Ekman 60 faces test revealed no differences in dwell time on the eyes (all P &gt; 0.05), but patients with bvAD (18.7 ± 9.5%) and bvFTD (19.4 ± 14.3%) spent significantly less dwell time on the mouth than individuals with SCD (30.7 ± 11.6%, P &lt; 0.01) and patients with tAD (32.7 ± 12.1%, P &lt; 0.01). Patients with bvAD (11.3 ± 4.6) exhibited lower scores on the Interpersonal Reactivity Index compared with individuals with SCD (15.6 ± 3.1, P = 0.05) and similar scores to patients with bvFTD (8.7 ± 5.6, P = 0.19) and tAD (13.0 ± 3.2, P = 0.43). The galvanic skin response to empathy eliciting videos did not differ between groups (all P &gt; 0.05). Patients with bvAD (16.0 ± 1.6) and bvFTD (15.2 ± 2.2) showed lower scores on the Social Norms Questionnaire than patients with tAD (17.8 ± 2.1, P &lt; 0.05) and individuals with SCD (18.3 ± 1.4, P &lt; 0.05). No group differences were observed in scores on moral dilemmas (all P &gt; 0.05), while only patients with bvFTD (0.9 ± 1.1) showed a lower galvanic skin response during personal dilemmas compared with SCD (3.4 ± 3.3 peaks per min, P = 0.01). Concluding, patients with bvAD showed a similar although milder social cognition profile and a similar eye-tracking signature to patients with bvFTD and greater social cognition impairments and divergent eye movement patterns compared with patients with tAD. Our results suggest reduced attention to salient facial features in these phenotypes, potentially contributing to their emotion recognition deficits.</p

CSF β-amyloid predicts prognosis in patients with multiple sclerosis

Background: The importance of predicting disease progression in multiple sclerosis (MS) has increasingly been recognised, hence reliable biomarkers are needed. Objectives: To investigate the prognostic role of cerebrospinal fluid (CSF) Amyloid beta1-42 (A) levels by the determination of a cut-off value to classify patients in slow and fast progressors. To evaluate possible association with white (WM) and grey matter (GM) damage at early disease stages. Methods: Sixty patients were recruited and followed-up for three to five years. Patients underwent clinical assessment, CSF analysis to determine Aβ levels, and brain MRI (at baseline and after 1 year). T1-weighted volumes were calculated. T2-weighted scans were used to quantify WM lesion loads. Results: Lower CSF Aβ levels were observed in patients with a worse follow-up EDSS (r=−0.65, p0.05). Conclusions: Low CSF Aβ levels may represent a predictive biomarker of disease progression in MS

CSF &#946;-amyloid predicts prognosis in patients with multiple sclerosis

Background: The importance of predicting disease progression in multiple sclerosis (MS) has increasingly been recognized, and hence reliable biomarkers are needed. Objectives: To investigate the prognostic role of cerebrospinal fluid (CSF) amyloid beta 1\u201342 (A\u3b2) levels by the determination of a cut-off value to classify patients in slow and fast progressors. To evaluate possible association with white matter (WM) and grey matter (GM) damage at early disease stages. Methods: Sixty patients were recruited and followed up for 3\u20135 years. Patients underwent clinical assessment, brain magnetic resonance imaging (MRI; at baseline and after 1 year), and CSF analysis to determine A\u3b2 levels. T1-weighted volumes were calculated. T2-weighted scans were used to quantify WM lesion loads. Results: Lower CSF A\u3b2 levels were observed in patients with a worse follow-up Expanded Disability Status Scale (EDSS; r = 120.65, p &lt; 0.001). The multiple regression analysis confirmed CSF A\u3b2 concentration as a predictor of patients\u2019 EDSS increase (r = 120.59, p &lt; 0.0001). Generating a receiver operating characteristic curve, a cut-off value of 813 pg/mL was determined as the threshold able to identify patients with worse prognosis (95% confidence interval (CI): 0.690\u20130.933, p = 0.0001). No differences in CSF tau and neurofilament light chain (NfL) levels were observed (p &gt; 0.05). Conclusion: Low CSF A\u3b2 levels may represent a predictive biomarker of disease progression in MS

Mindaugas e Radvilas

Desde 1920, quando enviou uma delegação pela primeira vez aos Jogos Olímpicos, o Brasil já foi representado por 2.111 atletas. Parte deles nasceu fora do país e é brasileira nata, por questões de ancestralidade familiar, ou naturalizada. Este texto discorre sobre Radvilas Gorauskas, que nasceu na Lituânia, imigrou para o Brasil no fim da década de 1940 e atuou como pivô pela seleção brasileira de basquete nos Jogos Olímpicos de Munique, em 1972. Refere-se ainda a Mindaugas, irmão de Radvilas, também nascido na Lituânia e atleta da seleção brasileira de basquete, por meio de quem a história do membro olímpico da família pode ser resgatada. O objetivo desse artigo é recuperar a memória da carreira esportiva de Radvilas, contextualizando todo o processo de deslocamento e a imigração desencadeada pela Segunda Guerra Mundial da família Gorauskas. O método utilizado baseia-se nas narrativas biográficas, e a entrevista com Mindaugas é a principal referência para a realização do trabalho, além de objetos biográficos trazidos pelo entrevistado. Conclui-se que a recuperação de um universo informativo é possível quando um narrador ainda vivo compartilha suas memórias. A entrevista com Mindaugas revelou dados pouco ou nada conhecidos não apenas sobre o processo migratório da família Gorauskas para o Brasil, como também do basquetebol das décadas de 1960 e 1970