Introduction: Currently there is no tool to quantify buccophonatory apraxia to stratify, compare and monitor patients longitudinally in an objective manner. Our aim in this study is to create a quantitative scale for buccophonatory apraxia and evaluate it in patients with the non-fluent/grammatical variant of Primary Progressive Aphasia (nfvPPA) and other neurodegenerative diseases that occur with speech and/or language problems. Methods: The scale was designed based on useful elements in the assessment of buccophonatory apraxia and the total was quantified in seconds. The scale was administered to 64 participants with diagnoses of: nfvPPA, semantic variant of Primary Progressive Aphasia (svPPA), logopenic variant of Primary Progressive Aphasia (lvPPA), Huntington's disease, Parkinson's disease, as well as a group of healthy controls. Results: Patients showed a significantly higher score compared to controls. The group nfvPPA had the highest mean score on the scale (429 seconds +/- 278). The scale was useful to differentiate vnfPPA from svPPA and Parkinson's disease (area under curve [AUC] of 0.956 and 0.989 respectively), but less to differentiate it from Huntington's disease (AUC = 0.67) and lvPPA. There was a statistically significant relationship between total score and disease severity in nfvPPA (p < 0.029). Conclusions: The Barcelona scale for buccophonatory apraxia could be useful to quantitatively evaluate buccophonatory apraxia in different neurodegenerative diseases, and compare patients, especially in nfvPPA. (c) 2022 Sociedad Espanola de Neurolog & imath;a. Published by Elsevier Espana, S.L.U. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/ 4.0/).

Background: Healthcare education programs have been studied in several chronic conditions, but evidence is lacking in multiple system atrophy (MSA). Here we present the definitive results of a study on satisfaction, quality of life and safety in people with MSA (PwMSA) and their caregivers after a comprehensive healthcare education program.

The role of the basal ganglia has been a longstanding issue in neural language models. Huntington's disease (HD) shows primary impairment in the striatum and has previously been shown to affect the processing of phrase-structural hierarchies that are built by phrasal movement (e.g. in passives). Here we asked patients with HD to judge the acceptability of sentences containing different types of illicit phrasal movement, which were contrasted with semantic violations involving no movement. A logistic mixed-effects regression showed that patients had a profound impairment in judging incorrect but not correct sentences across all types of illicit movement, while the semantic condition was also affected, but significantly less so. Adding neuropsychological variables to the model did not improve predictions. These results demonstrate a loss of cognitive control, worsening with disease progression, over phrase-structural hierarchies, which extends to forms of meaning built at sentential levels.

Objectives To conduct brainstem MRI shape analysis across neurodegenerative parkinsonisms and control subjects (CS), along with its association with clinical and cerebrospinal fluid (CSF) correlates. Methodology We collected demographic and clinical variables, performed planimetric and shape MRI analyses, and determined CSF neurofilament-light chain (NfL) levels in 84 participants: 11 CS, 12 with Parkinson’s disease (PD), 26 with multiple system atrophy (MSA), 21 with progressive supranuclear palsy (PSP), and 14 with corticobasal degeneration (CBD). Results MSA featured the most extensive and significant brainstem shape narrowing (that is, atrophy), mostly in the pons. CBD presented local atrophy in several small areas in the pons and midbrain compared to PD and CS. PSP presented local atrophy in small areas in the posterior and upper midbrain as well as the rostral pons compared to MSA. Our findings of planimetric MRI measurements and CSF NfL levels replicated those from previous literature. Brainstem shape atrophy correlated with worse motor state in all parkinsonisms and with higher NfL levels in MSA, PSP, and PD. Conclusion Atypical parkinsonisms present different brainstem shape patterns which correlate with clinical severity and neuronal degeneration. In MSA, shape analysis could be further explored as a potential diagnostic biomarker. By contrast, shape analysis appears to have a rather limited discriminant value in PSP. Key Points • Atypical parkinsonisms present different brainstem shape patterns . • Shape patterns correlate with clinical severity and neuronal degeneration . • In MSA, shape analysis could be further explored as a potential diagnostic biomarker .

INTRODUCTION:Differential diagnosis between Parkinson's disease (PD) and atypical parkinsonisms (APs: multiple system atrophy[MSA], progressive supranuclear palsy[PSP], corticobasal degeneration[CBD]) remains challenging. Lately, cerebrospinal fluid (CSF) studies of neurofilament light-chain (NFL) and RT-QuIC of alpha-synuclein (α-SYN) have shown promise, but data on their combination with MRI measures is lacking. OBJECTIVE:(1) to assess the combined diagnostic ability of CSF RT-QuIC α-SYN, CSF NFL and midbrain/pons MRI planimetry in degenerative parkinsonisms; (2) to evaluate if biomarker-signatures relate to clinical diagnoses and whether or not unexpected findings can guide diagnostic revision. METHODS:We collected demographic and clinical data and set up α-SYN RT-QuIC at our lab in a cross-sectional cohort of 112 participants: 19 control subjects (CSs), 20PD, 37MSA, 23PSP, and 13CBD cases. We also determined CSF NFL by ELISA and, in 74 participants (10CSs, 9PD, 26MSA, 19PSP, 10CBD), automatized planimetric midbrain/pons areas from 3T-MRI. RESULTS:Sensitivity of α-SYN RT-QuIC for PD was 75% increasing to 81% after revisiting clinical diagnoses with aid of biomarkers. Sensitivity for MSA was 12% but decreased to 9% with diagnostic revision. Specificities were 100% against CSs, and 89% against tauopathies raising to 91% with diagnostic revision. CSF NFL was significantly higher in APs. The combination of biomarkers yielded high diagnostic accuracy (PD vs. non-PD AUC = 0.983; MSA vs. non-MSA AUC = 0.933; tauopathies vs. non-tauopathies AUC = 0.924). Biomarkers-signatures fitted in most cases with clinical classification. CONCLUSIONS:The combination of CSF NFL, CSF RT-QuIC α-SYN and midbrain/pons MRI measures showed high discriminant ability across all groups. Results opposite to expected can assist diagnostic reclassification.

Resumen: Introducción: Actualmente no existe una herramienta que permita cuantificar la apraxia bucofonatoria para estratificar, comparar y monitorizar longitudinalmente a los pacientes de manera objetiva. Nuestro objetivo en el presente trabajo es crear una escala cuantitativa para la apraxia bucofonatoria y evaluarla en pacientes con la variante no fluente/agramatical de la afasia progresiva primaria (vnfAPP) y otras enfermedades neurodegenerativas que cursan con problemas del habla y/o lenguaje. Métodos: Se diseñó una escala a partir de elementos útiles en la exploración de la apraxia bucofonatoria y se cuantificó el total en segundos. Se administró la escala a 64 participantes con diagnósticos de vnfAPP, variante semántica de la afasia progresiva primaria (vsAPP), variante logopénica de la afasia progresiva primaria (vlAPP), enfermedad de Huntington, enfermedad de Parkinson, así como a un grupo de controles sanos. Resultados: Los pacientes mostraron una puntuación significativamente mayor respecto a los controles. El grupo de vnfAPP presentó la puntuación media más alta en la escala (429 ± 278 segundos). La escala resultó útil para diferenciar la vnfAPP de la vsAPP y de la enfermedad de Parkinson (área bajo la curva [AUC] de 0,956 y 0,989 respectivamente), pero menos para diferenciarla de la enfermedad de Huntington (AUC = 0,67) y de la vlAPP (AUC = 0,772). Existió una relación estadísticamente significativa entre la puntuación total y la gravedad de la enfermedad en la vnfAPP (p < 0,029). Conclusiones: La escala Barcelona para la apraxia bucofonatoria podría ser útil para evaluar cuantitativamente la apraxia bucofonatoria en diferentes enfermedades neurodegenerativas, y comparar pacientes, en especial en la vnfAPP. Abstract: Introduction: Currently there is no tool to quantify buccophonatory apraxia to stratify, compare and monitor patients longitudinally in an objective manner. Our aim in this study is to create a quantitative scale for buccophonatory apraxia and evaluate it in patients with the non-fluent/grammatical variant of Primary Progressive Aphasia (nfvPPA) and other neurodegenerative diseases that occur with speech and/or language problems. Methods: The scale was designed based on useful elements in the assessment of buccophonatory apraxia and the total was quantified in seconds. The scale was administered to 64 participants with diagnoses of: nfvPPA, semantic variant of Primary Progressive Aphasia (svPPA), logopenic variant of Primary Progressive Aphasia (lvPPA), Huntington's disease, Parkinson's disease, as well as a group of healthy controls. Results: Patients showed a significantly higher score compared to controls. The group nfvPPA had the highest mean score on the scale (429 seconds ± 278). The scale was useful to differentiate vnfPPA from svPPA and Parkinson's disease (area under curve [AUC] of 0.956 and 0.989 respectively), but less to differentiate it from Huntington's disease (AUC = 0.67) and lvPPA. There was a statistically significant relationship between total score and disease severity in nfvPPA (p < 0.029). Conclusions: The Barcelona scale for buccophonatory apraxia could be useful to quantitatively evaluate buccophonatory apraxia in different neurodegenerative diseases, and compare patients, especially in nfvPPA.

IntroductionHepatitis C virus (HCV) infection is known to be associated with neuropsychiatric manifestations as part of the disease. Previous neuroimaging studies showed brain connectivity dysfunction among HCV-infected patientsObjectivesTo assess, by MR in resting state, the potential structural and connectivity changes before (BL) and after HCV eradication (FU12) with direct-acting antivirals (DAA), along with clinical parameters.Methods Twenty-one HCV-patients, aged≤55 years, without psychiatric history, nor advanced liver disease, and eligible for DAA, and 25 healthy controls were included. Evaluations were performed at BL and FU12. Brain volume and local gyrification index (LGI) were assessed in MR-T1, and functional connectivity by seed-based analysis (left insula). Depression (MADRS/PHQ9) and neurotoxicity symptomatology (NRS) were assessed. We compared patients between BL/FU12, and controls by means of paired/independent T-test analysis.ResultsSubstained virologycal response was obtained in all patients (100%). Depressive and neurotoxicity symptomatology improved after cure (p<0.01). HCV-patients showed a reduced volume in a right latero-occipital area compared to controls (CWP<0.005) in both BL and FU12. This difference was smaller between FU12 and controls. LGI was higher in FU12-HCV compared to BL-HCV. fMRI connectivity showed a high association between insula and occipital/parietal territories in patients than controls, being higher among BL-HCV and controls. Differences were limited to occipital areas among FU12-HCV and controls.ConclusionsNeuropsychiatric symptomatology improved after cure. Left insula is altered among HCV-patients in structured and connectivity (mainly occipital areas). After cure differences with controls were reduced, suggesting a partial restoration of brain connectivity.

Urinary tract infections (UTIs) are the most common infections in renal transplant recipients (RTR). Klebsiella spp is a well-recognized source of nosocomial infections in immunocompromised patients and is also the most common pathogen capable of producing extended-spectrum β-lactamases (ESBLs).We performed a retrospective cohort study reviewing medical records of patients followed-up at Gdańsk Transplantation Centre. We analyzed urine cultures performed within the first 12 months after renal transplantation (RT) with reference to clinical data. We recorded all Klebsiella spp UTIs.We studied urine cultures and clinical data from 335 RTRs. We observed 59 Klebsiella spp episodes in 24 RT patients, including 10 cases of acute graft pyelonephritis and 8 of urosepsis. More than half were caused by ESBL+, whereas there were no carbapenemase-producing strains. Almost 80% of episodes were diagnosed beginning from the second month post-transplantation. More than 60% of upper Klebsiella spp UTIs were due to ESBL+ strains, although we did not identify any host risk factors including vesico-ureteral reflux, strictures at the uretero-vesical junction, history of recurrent UTIs before RT, comorbidity measured by Charlson Comorbidity Index, history of acute rejection, use of induction, and type of immunosuppression used. Upper Klebsiella spp UTIs were slightly more prevalent in males with urinary flow impairment due to various reasons.Klebsiella spp virulence factors, not the host factors, seem to be mostly responsible for developing upper UTIs in RT patients.

Abstract Background The N-methyl-D-aspartate receptor (NMDAR) hypofunction model of schizophrenia suggests that dysfunction of these receptors could underlie the brain functional abnormalities characterising these patients. In NMDAR encephalitis (NMDARE), which holds clinical similarities with schizophrenia, autoantibodies target NMDARs, predominantly located in the hippocampal region, leading to disrupted glutamatergic transmission. One study so far has described abnormal connectivity between the medial temporal lobe and posterior default mode network regions in patients with NMDARE (Peer et al., 2017), however no study so far has examined brain functional correlates of schizophrenia and NMDARE comparatively. Methods Patients with schizophrenia (N=16) and with NMDARE shortly after clinical stabilisation (N=15) were recruited within a tertiary setting, and compared with age and sex-matched healthy volunteers (N=20). All individuals were scanned with a 3T Siemens scanner including a functional resting state sequence, during which individuals were instructed to view a fixation cross. A seed-based analysis was performed using a spherical seed of 4mm located in the left hippocampus (MNI coordinates x =-32, y=-24, x=-14). Only grey matter voxels were considered to obtain the seed average signal. BOLD signal was preprocessed including slice timing, motion correction, spatial smoothing, and frequency filtering, and regressed taken into account movement parameters (rigid transformation, frame displacement, and DVARS). Statistics was performed between the correlation maps including age and sex as covariates. Family-wise error (FWE) was used to correct for multiple comparisons. Results Seed to voxel analyses revealed connectivity between the seed and the bilateral thalamus, parahippocampus, precuneus, posterior cingulate, right hippocampus and lateral temporal regions (threshold Fisher’s z > 0.28). We first examined differences in connectivity between both patient groups combined and healthy volunteers, which revealed greater connectivity in patients than in controls between the left hippocampus and the left inferior parietal, postcentral and posterior cingulate gyri (pFWE< 0.05). When examining patient groups individually, patients with schizophrenia continued to exhibit significantly greater connectivity between the left hippocampus and left inferior parietal and postcentral region (pFWE< 0.05) and at near trend level in the posterior cingulate (pFWE= 0.15). NMDARE patients also presented near trend level increased connectivity in the posterior cingulate and postcentral gyri (pFWE= 0.10). There were no differences in functional connectivity of the left hippocampus between patients with schizophrenia and with NMDARE. Discussion To our knowledge, this is the first study to compare patients with schizophrenia with patients with NMDARE using measures of brain function. We found that, for both conditions, the left hippocampus showed greater connectivity with areas belonging to the posterior default mode network. Connectivity between these regions has been associated with psychotic symptoms in schizophrenia (Lefebvre et al., 2016). Our findings suggest that this alteration could also underlie neuropsychiatric symptoms in NMDARE, and provides further evidence that NMDAR dysfunction may underpin the pathophysiology of schizophrenia.

Background: In multiple system atrophy (MSA) non-pharmacological therapeutic education of patients and caregivers is important due to its multisystem involvement. There are few published studies on healthcare education programs in MSA, with small samples, short follow-up and usually focused on a single intervention. We aimed at assessing perceived satisfaction of MSA patients and caregivers after a comprehensive healthcare education program.