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Background A central construct in Schema Therapy (ST) is that of a schema mode, describing the current emotional-cognitive-behavioral state. Initially, 10 modes were described. Over time, with the world-wide increasing and broader application of ST to various disorders, additional schema modes were identified, mainly based on clinical impressions. Thus, the need for a new, theoretically based, cross-cultural taxonomy of modes emerged. Methods An international workgroup started from scratch to identify an extensive taxonomy of modes, based on (a) extending the theory underlying ST with new insights on needs, and (b) recent research on ST theory supporting that modes represent combinations of activated schemas and coping. Results We propose to add two emotional needs to the original five core needs that theoretically underpin the development of early maladaptive schemas (EMSs), i.e., the need for Self-Coherence, and the need for Fairness, leading to three new EMSs, i.e. Lack of a Coherent Identity, Lack of a Meaningful World, and Unfairness. When rethinking the purpose behind the different ways of coping with EMS-activation, we came up with new labels for two of those: Resignation instead of Surrender, and Inversion instead of Overcompensation. By systematically combining EMSs and ways of coping we derived a set of schema modes that can be empirically tested. Conclusions With this project, we hope to contribute to the further development of ST and its application across the world.

The Sexual Inhibition Scales and Sexual Excitation Scales (Janssen et al., 2002a), based on the dual control model by Bancroft and Janssen (2000), are part of a 45-item self-report questionnaire evaluating individual tendencies to sexual inhibition or excitation according to three factors: two inhibition factors, SIS1, threat of performance failure, and SIS2, threat of performance consequences, and one excitation factor, SES. In this paper, we aimed to validate and explore psychometric properties of the SIS/SES in a sample of 2260 Italian men and women aged 18 to 75 years. Confirmatory factor analyses showed that the three-factor structure proposed in the original version of the scales fit with our sample. Moreover, our data confirmed the results of the original validation sample: Women scored higher on the SIS and lower on the SES than men did, but no significant differences appeared in the factor scores by age group, except for a gender × age interaction, where younger women had higher SIS2 scores. The SIS/SES appeared to be an effective, appropriate cross-cultural measurement of human sexuality in Italian samples, also shedding light on sexual arousal differences in women and men in our country. We also discuss clinical and therapeutic aspects.

Single reports of Guillain-Barré syndrome (GBS) have been reported worldwide during the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic. While case reports are likely to be biased toward uncommon clinical presentations, systematic assessment of prospective series can highlight the true clinical features and spectrum. In this prospective, observational study, we included all consecutive patients who developed GBS. In patients with SARS-CoV-2 infection as antecedent, the time-gap between the infection and GBS onset had to be ≤30 days. The referral was a neurological University Research Hospital, in the Italian Region more severely involved by the pandemic, and hospitalizing both COVID+ and non-COVID neurological diseases. Clinical, laboratory, cerebrospinal fluid, and electromyographic features of GBS diagnosed between March 2020 and March 2021 were compared to a retrospective series of GBS diagnosed between February 2019 and February 2020 (control population). Nasopharyngeal swab was still positive at GBS onset in 50% of patients. Mild-to-moderate COVID-related pneumonia, as assessed by X-ray (6 patients) or X-ray plus computerized tomography (2 patients) co-occurred in 6 of 10 patients. GBS diagnosed during the pandemic period, including 10 COVID-GBS and 10 non-COVID-GBS, had higher disability on admission (P = .032) compared to the GBS diagnosed between February 2019 and 2020, possibly related to later hospital referral in the pandemic context. Compared to non-COVID-GBS (n = 10) prospectively diagnosed in the same period (March 2020-2021), post-COVID-GBS (n = 10) had a higher disability score on admission (P = .028), lower sum Medical Research Council score (P = .022) and lymphopenia (P = .025), while there were no differences in GBS subtype/variant, severity of peripheral involvement, prognosis and response to treatment. Cerebrospinal fluid search for SARS-CoV-2 RNA and antiganglioside antibodies were negative in all COVID+ patients. Temporal clustering of cases, coinciding with the waves of the pandemic, and concomitant reduction of the incidence of COVID-negative GBSs may indicate a role for SARS-CoV-2 infection in the development of GBS, although the association may simply be related to a bystander effect of systemic inflammation; lack of prevalence of specific GBS subtypes in post-COVID-GBS also support this view. GBS features and prognosis are not substantially different compared to non-COVID-GBS.

Pneumatization variants of the temporal bone have a crucial importance in several surgical interventions. However, very few data are known about possible correlation with other pneumatization variants.Pneumatization of glenoid fossa, petrous apex, and infralabyrinthine portion of temporal bone was assessed in 200 computed tomography scans, equally divided between males and females (18-92 years). Pneumatization variants of the ethmoid (concha bullosa, agger nasi, pneumatized crista galli) and the sphenoid sinuses (pneumatized pterygoid processes, anterior clinoid processes, dorsum sellae, volume) were recorded as well.Differences in prevalence of each pneumatization type according to sex and side, among different portions of the temporal bone, and between temporal bone and the pneumatized variants of the ethmoid bone and sphenoid sinuses, were assessed through chi-square test (P

BackgroundIn women at high/intermediate lifetime risk of breast cancer (BC-LTR), contrast-enhanced magnetic resonance imaging (MRI) added to mammography ±ultrasound (Mx±US) increases sensitivity but decreases specificity. An alternative strategy, MRI alone, potentially more cost-effective, has never been explored. This study aims to assess the characteristics of women who participated in a randomized trial offering MRI alone.MethodsIn this feasibility multicentre randomized controlled trial we compared MRI alone versus Mx+US in women at intermediate BC-risk (allocation ratio 1:1). Eligible women were aged 40 –59, with a 15–30% LTR and/or extremely dense breasts. Two screening rounds per woman were planned in ten centres experienced in MRI screening. Primary endpoint: rate of cancers detected in the two arms after 5 years of follow-up. Secondary endpoints: distribution of the risk profiles among the women enrolled in the trial; distribution of pathological stages and histology of cancers detected; interval cancer rate in the two arms.ResultsFrom 07/2013 to 11/2015, 1,254 women (mean age 47 years) were enrolled: 624 were assigned to Mx+US, 630 to MRI. Most of them were aged below 50 (72%) and premenopausal (45%), 52% used oral contraceptives. Among postmenopausal women, 15% had used hormone replacement therapy. Breast and/or ovarian cancer in mother and/or sisters were reported by 37% of enrolled women, extreme breast density was recorded for 79%, 41% had a 15–30% BC-LTR.ConclusionsThe distribution of BC-risk profile major determinants (breast density and family of breast and ovarian cancer) of enrolled women varied across centres.Trial registrationNCT02210546Key pointsMammography plus ultrasound are commonly used to screen women with dense breastsSupplemental ultrasound increases BC detection rate but also false-positives and potential overdiagnosisWhether increased BC detection by US translates into reduced mortality is unknownMRI alone could be more risk- and cost-effective than mammography plus ultrasound

Purpose: To assess the ability of radiomic features (RF) extracted from contrast-enhanced CT images (ceCT) and non-contrast-enhanced (non-ceCT) in discriminating histopathologic characteristics of pancreatic neuroendocrine tumors (panNET). Methods: panNET contours were delineated on pre-surgical ceCT and non-ceCT. First- second- and higher-order RF (adjusted to eliminate redundancy) were extracted and correlated with histological panNET grade (G1 vs G2/G3), metastasis, lymph node invasion, microscopic vascular infiltration. Mann–Whitney with Bonferroni corrected p values assessed differences. Discriminative power of significant RF was calculated for each of the end-points. The performance of conventional-imaged-based-parameters was also compared to RF. Results: Thirty-nine patients were included (mean age 55-years-old; 24 male). Mean diameters of the lesions were 24 × 27mm. Sixty-nine RF were considered. Sphericity could discriminate high grade tumors (AUC = 0.79, p = 0.002). Tumor volume (AUC = 0.79, p = 0.003) and several non-ceCT and ceCT RF were able to identify microscopic vascular infiltration: voxel-alignment, neighborhood intensity-difference and intensity-size-zone families (AUC ≥ 0.75, p < 0.001); voxel-alignment, intensity-size-zone and co-occurrence families (AUC ≥ 0.78, p ≤ 0.002), respectively). Non-ceCT neighborhood-intensity-difference (AUC = 0.75, p = 0.009) and ceCT intensity-size-zone (AUC = 0.73, p = 0.014) identified lymph nodal invasion; several non-ceCT and ceCT voxel-alignment family features were discriminative for metastasis (p < 0.01, AUC = 0.80–0.85). Conventional CT ‘necrosis’ could discriminate for microscopic vascular invasion (AUC = 0.76, p = 0.004) and ‘arterial vascular invasion’ for microscopic metastasis (AUC = 0.86, p = 0.001). No conventional-imaged-based-parameter was significantly associated with grade and lymph node invasion. Conclusions: Radiomic features can discriminate histopathology of panNET, suggesting a role of radiomics as a non-invasive tool for tumor characterization. Trial registration number: NCT03967951, 30/05/2019

Chest X-ray (CXR) is an essential first-line tool in COVID-19 pneumonia diagnosis and management. Our study aimed at assessing 1) CXR manifestations, frequency, and distribution; 2) the feasibility and repeatability of a CXR severity score; and 3) the correlation between the CXR severity score and clinical and laboratory parameters. We reviewed baseline CXRs and clinical data of consecutive patients who presented to our emergency department and resulted positive at SARS-CoV-2 reverse transcriptase-PCR oropharyngeal swab test from March 1, 2020 to April 6, 2020. Lung abnormalities and their distribution were analyzed. A score of CXR severity was assigned by two radiologists, independently, according to the extent of lung involvement, with a maximum score of 8 for CXR. Correlations between the CXR score and the clinical data were assessed. One hundred fifty-five patients were included; 143/155 (92%) were positive at baseline CXR. Ground-glass opacity was the most common finding (141/143, 99%). Involvement was mainly bilateral (96/143, 67%), with peripheral distribution (79/143, 55%). The mean CXR severity score was 3.3 (±2); interobserver agreement was excellent, with a Cohen's K correlation coefficient of 0.901. The CXR score showed a significant positive correlation with C-reactive protein, lactate dehydrogenase, and fever duration, and a negative correlation with oxygen saturation. Chest X-ray findings are in line with those reported by computed tomography studies. The use of a visual CXR score, easy to assess and highly reproducible, can reflect the clinical severity and help the patients' management.

AimTo assess the most common chest X-Ray findings and distribution in patients with confirmed diagnosis of COVID-19; to verify the repeatability of a radiological severity score, based on visual quantitative assessment; to assess the evolution of chest X-Ray findings at follow-up; to evaluate chest X-Ray sensitivity.MethodsWe analysed chest X-Rays at baseline of 110 consecutive COVID-19 patients (79 males, 31 females; mean age: 64±16 years) with RT-PCR confirmation, who presented to our ED.Two radiologists evaluated the imaging findings and distribution.A severity score, based on the extension of lung abnormalities, was assigned by two other radiologists, independently, to the baseline and follow-up X-Rays, executed in 77/110 cases; interobserver agreement was calculated. Chest X-Ray sensitivity was assessed, with RT-PCR as gold standard.ResultsInterobserver agreement was excellent for baseline and follow-up X-Rays (Cohen's K=0.989, p

BACKGROUND Invasive imaging techniques have been applied for lymphedema (LE) assessment; noncontrast MR lymphography (NCMLR) has potential as an alternative, but its performance is not known in secondary lower limb LE. PURPOSE To assess the role of NCMRL for the classification and characterization of secondary lower limb LE. STUDY TYPE Retrospective. POPULATION Fifty adults with clinically diagnosed secondary LE. FIELD STRENGTH/SEQUENCE 1.5T, 3D T2 -weighted turbo spin-echo, 3D T2 -weighted turbo spin-echo short tau inversion recovery. ASSESSMENT Three radiologists assessed the following characteristics on NCMRL: honeycomb pattern, dermal thickening, muscular abnormalities, distal dilated lymphatics, inguinal lymph node number, appearance of iliac lymphatic trunks. An LE grading based on the MR images was assigned. The relationship between imaging findings and clinical staging was evaluated, as well as between dermal backflow at lymphoscintigraphy and MR staging, and between the limb swelling duration and peripheral lymphatics dilatation. STATISTICAL TESTS Pearson's correlation test and Cramer's V coefficient were computed to measure the strength of association. The Mann-Whitney test was used to compare the limb swelling duration between patients with and without dilated distal vessels. Agreement among raters was assessed through Kendall's W coefficient of correlation. RESULTS Clinical stage and the MR grading were correlated, with Cramer's V coefficient of 1 for reader 1 (P

To assess imaging findings and characteristics of the lymphatic system in patients affected by lipedema and lipolymphedema of the lower extremities on Non-Contrast MR Lymphography (NCMRL).44 lower extremities in 11 consecutive female patients affected by lipedema, and 11 patients with lipolymphedema were examined by NCMRL. MR imaging was performed on 1.5-T system MR equipment. The examination consisted of one 3D short-tau inversion recovery (STIR) and one heavily T2-weighted 3D-Turbo Spin Echo (TSE) sequence.All patients showed symmetrical enlargement of the lower extremities with increased subcutaneous fat tissue. The fat tissue was homogeneous, without any signs of edema in pure lipedema patients. In all the extremities with lipolymphedema, high signal intensity areas in the epifascial region could be detected on the 3D-TSE sequence (p .001) with evidence of mild epifascial fluid collections (p .001). No sign of honeycomb pattern fat appearance was observed. The appearance of the iliac lymphatic trunks was normal in both lipedema and lipolymphedema patients. Dilated peripheral lymphatics were observed in 2 patients affected by lipedema, indicating a subclinical status of lymphedema, and in 10 patients with lipolymphedema (p = .001). Signs of vascular stasis were observed in both groups, without statistically significant difference (p = .665).NCMRL is a non-invasive imaging technique that is suitable for the evaluation of patients affected by lipedema and lipolymphedema, helping in the differential diagnosis.

This study was based on exploring the role of attachment style related to emotional abuse and sexual coercion in a sample of people who are gay and lesbian. A total of 182 young adults, from 18 to 25 years old (58% females, M = 22.42, Sd = 1.91), participated. Participants completed the multidimensional measure of emotional abuse to investigate emotional abuse (acted and suffered), the sexual coercion in intimate relationship scale (acted) to evaluate sexual coercion, and attachment style to investigate interpersonal attachment. The model shows that the secure attachment style is negatively connected with coercion of resources and violence, as well as acted emotional abuse. Discomfort with closeness is negatively connected with coercion of resources and violence. Need for approval is positively connected with coercion of resources and violence, as well as with manipulation. Preoccupation with relationships is negatively connected with manipulation and acted emotional abuse and is positively connected with commitment defection and suffered emotional abuse. Finally, relationship as being secondary is positively connected with suffered emotional abuse. Our results show that an inadequate attachment style in homosexual couples is responsible for sexual coercion and emotional abuse, both in males and females. Theoretical and clinical implications are discussed.

Anatomical uniqueness plays a significant role in the personal identification process of unknown deceased. Frontal sinuses have been widely used in the past decades for this purpose, mostly using 2D X-ray techniques. However, the modern 3D CT-based segmentation methods may help in developing novel and more reliable methods of identification. This study aims at assessing the anatomical uniqueness of frontal sinuses through the 3D model registration. Thirty subjects who underwent two maxillofacial CT scans (interval: 1 month to 5 years) were selected from a hospital database. Frontal sinuses were automatically segmented through ITK-SNAP open source software and the 3D models belonging to the same patient were automatically superimposed according to the least point-to-point difference between the two surfaces. Two hundred patients were randomly selected from the same database and undergo the same procedure to perform 200 superimpositions of frontal sinuses belonging to different individuals, equally divided between males and females (mismatches). Statistically significant differences of average root mean square (RMS) point-to-point distance between the group of matches and mismatches, as well as possible differences according to sex, were assessed through Mann-Whitney U test (p

To give an update on the most recent studies regarding the role of schema therapy in the treatment of emotion dysregulation related to personality disorders.In personality disorders, a lack of emotion regulation can be found. Schema therapy treats emotion dysregulation with a series of techniques, such as imagery rescripting, limited reparenting, chairwork, and cognitive restructuring to remove dysregulatory mechanism.Schema therapy is one of the most efficient therapies for personality disorders. However, there is a lack of recent studies on how it treats emotion dysregulation. Although the treatment of emotional dysregulation is not the core of schema therapy, it is certainly important inside this theoretical framework. The mode model helps clinicians address their work toward the reduction of dysfunctional modes, whereas fostering functional modes.

The management of orbital blowout fractures (BOFs) is controversial: the evaluation of diplopia is the most important criterion for planning whether to undertake surgery. Our aim was to determine CT findings that may suggest the presence of diplopia when patients with BOFs cannot be adequately examined to plan an orbital repair. We retrospectively evaluated CT of all patients presented to our Emergency Department for blunt craniofacial trauma (N = 3334) from January 2014 to March 2016, selecting patients with CT-demonstrated BOFs. The following CT variables were assessed: fracture location, fracture multifocality, bone fragments displacement, extraocular muscles (EOM) thickening, EOM entrapment, EOM displacement, EOM hooking, intraconal and extraconal emphysema, intraconal and extraconal hematoma, and fat herniation. All patients underwent Hess-Lancaster test, to establish the presence of diplopia. After performing group comparison with Pearson χ2 test, we derived our prediction model by using logistic regression, with diplopia as the prediction and CT variables as predictors. We observed 299 patients with BOFs, 46 (15.4%) with a Hess Lancaster test-proven diplopia. The CT variables with statistically significant difference between the group with diplopia and the group without diplopia were as follows: floor fracture (p = .014), bone fragments displacement (p = .001), multifocality (p = .005), EOM thickening (p = .001), EOM entrapment (p

Our aim was to assess MRI findings in the acute phase of ON and their correlation with visual acuity at presentation, visual outcome (VO) and MS development, to analyze a possible correlation between lesions number and diagnosis, and to assess correlation between orbits MRI and OCT.We retrospectively studied 37 patients, who presented to our Emergency Department with an ON first episode from January 2015 to January 2017. Patients underwent immediately a complete neuro-ophthalmological evaluation, blood test, CSF analysis. MRI of brain, orbits, cervical spine was executed within 7 days from ON onset. Brain MRI was classified as: normal, non-specific, suspected demyelination, lesions with dissemination in space and time. Optic nerves findings were localized in three sites (intra-orbital, canalicular and chiasmal) and classified as: normal, STIR- alteration, altered contrast enhancement. Patients underwent neuro-ophthalmological follow-up and MRI at 6 months to assess VO (complete recovery, partial recovery, deficit persistence). Another follow-up at 1 year was performed to identify MS or clinically isolated syndrome (CIS).64.8% patients received a diagnosis of MS; 35% of CIS. Lesions of the optic nerve were found in 65.8%. We observed statistically significant correlation between brain MRI pattern and diagnosis and between lesions number and diagnosis. We observed a statistically significant correlation between orbital MRI pattern and optical coherence tomography (OCT) results. MRI brain findings correlate with development of MS. MRI brain features and lesions number can predict the risk of MS conversion.

Our experience suggests that ultrasound-assisted thrombolysis with the EkoSonic system could be a valid bail-out strategy in cases of massive pulmonary embolism unresponsive to systemic thrombolysis and provides insight into the role of prolonged administration time in non-high bleeding risk patients with elevated thrombotic burden and initial suboptimal results. In this setting, multimodality imaging could guide patient selection and monitor treatment efficacy to individualize its duration in cases of slow hemodynamic improvement.

Background Squid, as Onyx, is an ethylene-vinyl alcohol copolymer (EVOH)-based liquid embolic agent developed for neuroradiologic interventions with poor application in abdominal district. Our aim was to evaluate safety, complications, and efficacy of transcatheter embolization using the two available formulations Squid-18 and 12, in 30 patients affected by different abdominal diseases. Results Transcatheter embolization with Squid, combined with other embolic agents, as poly vinyl alcohol (PVA) particles, coils and amplatzer plugs, or alone (type 2 endoleak), was performed in 30 patients, as follows: 10 portal vein embolizations (PVEs), 6 arteriovenous malformations (AVMs), 5 visceral artery aneurysms (VAAs), 4 type 2 endoleaks, 3 preoperative embolizations, 1 acute arterial bleeding, 1 female varicocele. Squid was always administered using dimethyl sulfoxide (DMSO) compatible microcatheters. Technical success, 30-day clinical success and complications were assessed. Technical success was 90%. 3 patients (2 AVMs, 1 VAA) required re-intervention successfully performed in all cases. Major complications, cases of microcatheter entrapment and DMSO-related poor pain control were not recorded. 30-day clinical success was 93.3%: in 2 patients submitted to PVE a sufficient future liver remnant (FLR) hypertrophy was not achieved. Conclusion Squid was successfully used with low complication rate in many abdominal diseases showing a valid embolic action either combined with other embolic agents or alone in type 2 endoleak. The availability of different formulations (Squid-18 and Squid-12) variable for viscosity makes Squid preferable to Onyx as EVOH-based liquid embolic agent, even though comparable studies in different abdominal districts with a larger cohort of patients will be necessary.

Schema therapy (ST) has been proven to be an effective psychotherapy model in the treatment of complex personality disorders. ST helps analyze causes of emotional, cognitive, and behavioral alteration in patients using schemas and modes (i.e., sets of emotional states, coping responses and schemas active in a given moment). ST finds its empirical validation in the short Schema Mode Inventory (SMI), a practical tool consisting of 14 subscales assessing 14 different mode categories, grouped in 4 (child, coping, parent and adult modes) high-order categories used to assess different modes at different times. We introduced the Italian validation of the short SMI to a sample of 707 participants, of whom 230 were psychiatric patients. Confirmatory factor analysis (CFA) provides evidence that the 14-dimensional model best describes the SMI’s structure at a lower level. Higher-order CFA provides evidence for both four higher-level mode categories and one higher-level mode category, the four-mode category being the best approach. Internal reliability, test–retest stability and the relationship between the SMI’s subscales have been evaluated with promising results. Clinical vs nonclinical subjects were compared with a multigroup CFA in order to test invariance and with a MANOVA and Bonferroni post hoc comparisons in order to test mean differences. A linear thread was found for all modes except Bully/Attack. While contributing to international research and to the diffusion of SMI and schema therapy, our results also suggest that SMI is a powerful tool for the assessment of modes in ST, both in therapeutic and diagnostic contexts.

Background: Spinocerebellar ataxias are rare dominantly inherited neurodegenerative diseases that lead to severe disability and premature death. Objective: To quantify the impact of disease progression measured by the Scale for the Assessment and Rating of Ataxia on survival, and to identify different profiles of disease progression and survival. Methods: Four hundred sixty-two spinocerebellar ataxia patients from the EUROSCA prospective cohort study, suffering from spinocerebellar ataxia type 1, spinocerebellar ataxia type 2, spinocerebellar ataxia type 3, and spinocerebellar ataxia type 6, and who had at least two measurements of Scale for the Assessment and Rating of Ataxia score, were analyzed. Outcomes were change over time in Scale for the Assessment and Rating of Ataxia score and time to death. Joint model was used to analyze disease progression and survival. Results: Disease progression was the strongest predictor for death in all genotypes: An increase of 1 standard deviation in total Scale for the Assessment and Rating of Ataxia score increased the risk of death by 1.28 times (95% confidence interval: 1.18–1.38) for patients with spinocerebellar ataxia type 1; 1.19 times (1.12–1.26) for spinocerebellar ataxia type 2; 1.30 times (1.19–1.42) for spinocerebellar ataxia type 3; and 1.26 times (1.11–1.43) for spinocerebellar ataxia type 6. Three subgroups of disease progression and survival were identified for patients with spinocerebellar ataxia type 1: “severe” (n = 13; 12%), “intermediate” (n = 31; 29%), and “moderate” (n = 62; 58%). Patients in the severe group were more severely affected at baseline with higher Scale for the Assessment and Rating of Ataxia scores and frequency of nonataxia signs compared to those in the other groups. Conclusion: Rapid ataxia progression is associated with poor survival of the most common spinocerebellar ataxia. Theses current results have implications for the design of future interventional studies of spinocerebellar ataxia. © 2019 International Parkinson and Movement Disorder Society., SCOPUS: ar.j, info:eu-repo/semantics/published

Purpose: The aim of this study was to quantify the impact of CT delineation uncertainty of pancreatic neuroendocrine neoplasms (panNEN) on Radiomic features (RF). Methods: Thirty-one previously operated patients were considered. Three expert radiologists contoured panNEN lesions on pre-surgical high-resolution contrast-enhanced CT images and contours were transferred onto pre-contrast CT. Volume agreement was quantified by the DICE index. After images resampling and re-binning, 69 RF were extracted and the impact of inter-observer variability was assessed by Intra-Class Correlation (ICC): ICC > 0.80 was considered as a threshold for “very high” inter-observer agreement. Results: The median volume was 1.3 cc (range: 0.2–110 cc); a satisfactory inter-observer volume agreement was found (mean DICE = 0.78). Only 4 RF showed ICC < 0.80 (0.48–0.73), including asphericity and three RFs (of five) of the neighborhood intensity difference matrix (NID). Conclusions: The impact of inter-observer variability in delineating panNEN on RF was minimum, with the exception of the NID family and asphericity, showing a moderate agreement. These results support the feasibility of studies aiming to assess CT radiomic biomarkers for panNEN.

Purpose Patients with Parkinson's disease (PD) variably report sexual dysfunctions. We assessed sexuality in PD by comparing sexual function between a large group of patients with idiopathic PD and a group of subjects without PD. Methods We recruited 121 patients with mild-to-moderate PD (aged 40–80 years) from four Italian Movement Disorder Clinics and 123 non-Parkinsonian controls (NPC) (aged 40–80 years). Sexual function was assessed with four scales: the Brief Index of Sexual Functioning (BISF-M for men; BISF-W for women), the International Index of Erectile Function (IIEF), and the Female Sexual Function Index (FSFI). Both groups also underwent assessment with the Beck Depression Inventory (BDI) and the Mini Mental State Examination (MMSE), and patients were assessed with the Parkinson's Disease Questionnaire-8 (PDQ-8). Results No differences in total score were found between PD and NPC for any sexual function scale (BISF-M, BISF-W, IIEF, FSFI: p > 0.05). However, the Orgasm/Pleasure Domain (BISF, D5) was significantly lower in male patients than in controls. Conclusion Our findings fail to confirm previous findings that PD is associated with a significant sexual impairment. NPC and patients with PD have comparable sexual function in both sexes. Thus, rather than dismissing sexual dysfunction as a normal parkinsonian symptom, physicians should refer patients to sexual medicine specialists who can investigate and discuss problems fully, diagnose possible comorbidities, and suggest appropriate treatments.

Item does not contain fulltext BACKGROUND: Spinocerebellar ataxias are dominantly inherited neurodegenerative diseases. As potential treatments for these diseases are being developed, precise knowledge of their natural history is needed. We aimed to study the long-term disease progression of the most common spinocerebellar ataxias: SCA1, SCA2, SCA3, and SCA6. Furthermore, we aimed to establish the order and occurrence of non-ataxia symptoms, and identify predictors of disease progression. METHODS: In this longitudinal cohort study (EUROSCA), we enrolled men and women with positive genetic testing for SCA1, SCA2, SCA3, or SCA6 and with progressive, otherwise unexplained ataxia who were aged 18 years or older from 17 ataxia referral centres in ten European countries. Patients were seen every year for 3 years, and at irregular intervals thereafter. The primary outcome was the scale for the assessment and rating of ataxia (SARA), and the inventory of non-ataxia signs (INAS). We used linear mixed models to analyse progression. To account for dropouts, we applied a pattern-mixture model. This study is registered with ClinicalTrials.gov, number NCT02440763. FINDINGS: Between July 1, 2005, and Aug 31, 2006, 526 patients with SCA1, SCA2, SCA3, or SCA6 were enrolled. We analysed data for 462 patients with at least one follow-up visit. Median observation time was 49 months (IQR 35-72). SARA progression data were best fitted with a linear model in all genotypes. Annual SARA score increase was 2.11 (SE 0.12) in patients with SCA1, 1.49 (0.07) in patients with SCA2, 1.56 (0.08) in patients with SCA3, and 0.80 (0.09) in patients with SCA6. The increase of the number of non-ataxia signs reached a plateau in SCA1, SCA2, and SCA3. In patients with SCA6, the number of non-ataxia symptoms increased linearly, but more slowly than in patients with SCA1, SCA2, and SCA3 (p

The pterygopalatine fossa is an important anatomical structure for several surgical and anaesthesiologic procedures; yet, very few data are available about its size. This study aims at providing a metrical assessment of pterygopalatine fossa through an innovative 3D segmentation procedure on head CT-scans. CT-scans from 100 patients (50 males and 50 females) aged between 18 and 85 years were chosen for the study. Right and left pterygopalatine fossae were segmented through ITK-SNAP open source software. Height and volume were calculated on the acquired 3D models. In addition, anterior–posterior nasal spine distance, upper facial height (nasion–prosthion) and biorbital breadth (ectoconchion–ectoconchion) were measured as well. Statistically significant differences of height and volume according to sex and side were assessed through two-way ANOVA test: sexually dimorphic measurements were further assessed through one-way ANCOVA test using the three cranial measurements as covariates (p 0.05); interaction was negligible for both the measurements. ANCOVA test verified that sexual dimorphism of both measurements is independent from general cranial size (p

To assess the amount of computed tomography (CT) scans for minor head injury (MHI) performed in young patients in our emergency department (ED), not indicated by National Institute for Health and Clinical Excellence (NICE) and Canadian Computed Tomography Head Rules (CCHR), and to analyze factors contributing to unnecessary examinations. Secondary objectives were to calculate the effective dose, to establish the number of positive CT and to analyze which of the risk factors are correlated with positivity at CT; finally, to calculate sensitivity and specificity of NICE and CCHR in our population. We retrospectively evaluated 493 CT scans of patients aged 18–45 years, collecting the following parameters from ED medical records: patient demographics, risk factors indicating the need of brain imaging, trauma mechanism, specialty and seniority of the referring physician. For each CT, the effective dose and the negativity/positivity were assessed. 357/493 (72%) and 347/493 (70%) examinations were not in line with the CCHR and NICE guidelines, respectively. No statistically significant difference between physician specialty (p = 0.29 for CCHR; p = 0.24 for NICE), nor between physician seniority and the amount of inappropriate examinations (p = 0.93 for CCHR, p = 0.97 for NICE) was found but CT scans requested by ED physicians were less inappropriate [p = 0.28, odds ratio (OR) 0.562, CI (95%) 0.336–0.939]. There was no statistically significant correlation between patient age and over-referral (p = 0.74 for NICE, p = 0.93 for CCHR). According to NICE, low speed motor vehicle accident (p = 0.009), motor vehicle accident with high energy impact (p

Aim Sella turcica bridging and ossified carotico-clinoid ligament are two variants of the sella turcica, the origin of which is partially unknown. These variations should be properly recognised, as they may hamper the removal of the anterior clinoid process in surgical procedures. Therefore, our aim was to determine the prevalence of these two anatomical variants and to investigate their prevalence according to patient sex and age in a series of maxilla computed tomography scans. Materials and methods We revised 300 computed tomography scans of the head from northern Italian patients, stratified into three age groups (18–40 years, 41–60 years, >60 years): a logistic regression analysis was used to explore an association of sella turcica bridging with age and sex through Matlab software, also including a test for the extracted model ( P Results The mean prevalence of sella turcica bridging and ossified carotico-clinoid ligament were 0.16 ± 0.06 (48/300, 16.0%) and 0.09 ± 0.03 (26/300, 8.7%), respectively. Statistically significant differences according to sex were found neither for sella turcica bridging ( P = 0.345) nor for ossified carotico-clinoid ligament ( P = 0.412). Only sella turcica bridging showed a correlation with age ( P = 0.007). In addition, the two variants were often associated, as patients without sella turcica bridging usually did not show ossified carotico-clinoid ligament ( P Discussion Our results suggest an association between the two variants, and provide a novel contribution to the debate around their origin.

Summary Background Spinocerebellar ataxias are dominantly inherited progressive ataxia disorders that can lead to premature death. We aimed to study the overall survival of patients with the most common spinocerebellar ataxias (SCA1, SCA2, SCA3, and SCA6) and to identify the strongest contributing predictors that affect survival. Methods In this longitudinal cohort study (EUROSCA), we enrolled men and women, aged 18 years or older, from 17 ataxia referral centres in ten European countries; participants had positive genetic test results for SCA1, SCA2, SCA3, or SCA6 and progressive, otherwise unexplained, ataxias. Survival was defined as the time from enrolment to death for any reason. We used the Cox regression model adjusted for age at baseline to analyse survival. We used prognostic factors with a p value less than 0·05 from a multivariate model to build nomograms and assessed their performance based on discrimination and calibration. The EUROSCA study is registered with ClinicalTrials.gov, number NCT02440763. Findings Between July 1, 2005, and Aug 31, 2006, 525 patients with SCA1 (n=117), SCA2 (n=162), SCA3 (n=139), or SCA6 (n=107) were enrolled and followed up. The 10-year survival rate was 57% (95% CI 47–69) for SCA1, 74% (67–81) for SCA2, 73% (65–82) for SCA3, and 87% (80–94) for SCA6. Factors associated with shorter survival were: dysphagia (hazard ratio 4·52, 95% CI 1·83–11·15) and a higher value for the Scale for the Assessment and Rating of Ataxia (SARA) score (1·26, 1·19–1·33) for patients with SCA1; older age at inclusion (1·04, 1·01–1·08), longer CAG repeat length (1·16, 1·03–1·31), and higher SARA score (1·15, 1·10–1·20) for patients with SCA2; older age at inclusion (1·44, 1·20–1·74), dystonia (2·65, 1·21–5·53), higher SARA score (1·26, 1·17–1·35), and negative interaction between CAG and age at inclusion (0·994, 0·991–0·997) for patients with SCA3; and higher SARA score (1·17, 1·08–1·27) for patients with SCA6. The nomogram-predicted probability of 10-year survival showed good discrimination ( c index 0·905 [SD 0·027] for SCA1, 0·822 [0·032] for SCA2, 0·891 [0·021] for SCA3, and 0·825 [0·054] for SCA6). Interpretation Our study provides quantitative data on the survival of patients with the most common spinocerebellar ataxias, based on a long follow-up period. These results have implications for the design of future interventional studies of spinocerebellar ataxias; for example, the prognostic survival nomogram could be useful for selection and stratification of patients. Our findings need validation in an external population before they can be used to counsel patients and their families. Funding European Union 6th Framework programme, German Ministry of Education and Research, Polish Ministry of Scientific Research and Information Technology, European Union 7th Framework programme, and Fondation pour la Recherche Medicale.

Purpose High accuracy in delineating pancreatic neuroendocrine tumors (NET) is a pre-requisite for the assessment of CT-based biomarkers, including radiomic features (RF). The aim of this study was to quantify inter-observer variability in delineating NET on CT images and its impact on RF. Methods Three radiologists contoured volumes of 32 NET patients. The contours were delineated using arterial or venous phase. The contours delineated on contrast enhanced images were overlayed onto CT images acquired before contrast injection, and possibly corrected to take into account small anatomical discrepancies. Inter-observer contouring variability was assessed by DICE index and volume agreement; paired analysis between observers was performed. Seventy-one RF (12 of first and 59 of higher order) were extracted for all contours, for each observer by using the CGITA software (v 1.4). Their robustness was investigated against contour variability by Spearman R values and intra-class correlation coefficients (ICC, taking the worst value of the tested pairs). RF were classified as optimal, good and modest according to their ICC: ⩾0.90, ⩾0.80 and Results The mean/median values of NET volumes were 14.7/1.3 cc. Despite the prevalence of small tumors, a satisfactory agreement was found (mean DICE = 0.78; SD 0.09) with no significant differences between observers. Sixty-three RF were classified as optimal and only 1 showed ICC 0.9 and 0.82 for the remaining ones. Examples of correlation for four different features (including asphericity) are shown in the Figure. Conclusions Results show a relatively small inter-observer variability in delineating pancreatic NET with little impact on RF. This finding suggests the possibility to robustly extract RF features in large pancreatic NET populations to assess CT-based radiomic biomarkers.

Summary Background Friedreich's ataxia is a rare autosomal recessive neurodegenerative disorder. Here we report cross-sectional baseline data to establish the biological and clinical characteristics for a prospective, international, European Friedreich's ataxia database registry. Methods Within the European Friedreich's Ataxia Consortium for Translational Studies (EFACTS) framework, we assessed a cohort of patients with genetically confirmed Friedreich's ataxia. The primary outcome measure was the Scale for the Assessment and Rating of Ataxia (SARA) and secondary outcome measures were the Inventory of Non-Ataxia Signs (INAS), the performance-based coordination test Spinocerebellar Ataxia Functional Index (SCAFI), the neurocognitive phonemic verbal fluency test, and two quality-of-life measures: the activities of daily living (ADL) part of the Friedreich's Ataxia Rating Scale and EQ-5D. The Friedreich's ataxia cohort was subdivided into three groups: early disease onset (≤14 years), intermediate onset (15–24 years), and late onset (≥25 years), which were compared for clinical characteristics and outcome measures. We used linear regression analysis to estimate the annual decline of clinical outcome measures based on disease duration. This study is registered with ClinicalTrials.gov, number NCT02069509. Findings We enrolled 592 patients with genetically confirmed Friedreich's ataxia between Sept 15, 2010, and April 30, 2013, at 11 sites in seven European countries. Age of disease onset was inversely correlated with the number of GAA repeats in the frataxin ( FXN ) gene: every 100 GAA repeats on the smaller repeat allele was associated with a 2·3 year (SE 0·2) earlier onset. Regression analyses showed significant estimated annual worsening of SARA (regression coefficient 0·86 points [SE 0·05], INAS (0·14 points [0·01]), SCAFI Z scores (−0·09 [0·01]), verbal fluency (−0·34 words [0·07]), and ADL (0·64 points [0·04]) during the first 25 years of disease; the regression slope for health-related quality-of-life state from EQ-5D was not significant (−0·33 points [0·18]). For SARA, the predicted annual rate of worsening was significantly higher in early-onset patients (n=354; 1·04 points [0·13]) and intermediate-onset patients (n=137; 1·17 points [0·22]) than in late-onset patients (n=100; 0·56 points [0·10]). Interpretation The results of this cross-sectional baseline analysis of the EFACTS cohort suggest that earlier disease onset is associated with larger numbers of GAA repeats and more rapid disease progression. The differential estimated progression of ataxia symptoms related to age of onset have implications for the design of clinical trials in Friedreich's ataxia, for which SARA might be the most suitable measure to monitor disease progression. Funding European Commission.

BackgroundSensitive outcome measures for clinical trials on cerebellar ataxias are lacking. Most cerebellar ataxias progress very slowly and quantitative measurements are required to evaluate cerebellar dysfunction.MethodsWe evaluated two scales for rating cerebellar ataxias: the Composite Cerebellar Functional Severity (CCFS) Scale and Scale for the Assessment and Rating of Ataxia (SARA), in patients with spinocerebellar ataxia (SCA) and controls. We evaluated these scales for different diseases and investigated the factors governing the scores obtained. All patients were recruited prospectively.ResultsThere were 383 patients with Friedreich’s ataxia (FRDA), 205 patients with SCA and 168 controls. In FRDA, 31% of the variance of cerebellar signs with the CCFS and 41% of that with SARA were explained by disease duration, age at onset and the shorter abnormal repeat in the FXN gene. Increases in CCFS and SARA scores per year were lower for FRDA than for SCA (CCFS index: 0.123±0.123 per year vs 0.163±0.179, PConclusionsCerebellar dysfunction, as measured with the CCFS and SARA scales, was more severe in FRDA than in patients with SCA, but with lower progression indexes, within the limits of these types of indexes. Ceiling effects may occur at late stages, for both scales. The CCFS scale is rater-independent and could be used in a multicentre context, as it is simple, rapid and fully automated.Trial registration numberClinicalTrials.gov: NCT02069509.

Objective: Human cancers display intra-tumor phenotypic heterogeneity and recent research has focused on developing image processing methods extracting imaging descriptors to characterize this heterogeneity. This work assesses the role of pretreatment 18F-FDG PET and DWI-MR with respect to the prognosis and prediction of neoadjuvant chemotherapy (NAC) outcomes when image features are used to characterize primitive lesions from breast cancer (BC). Materials and methods: A retrospective protocol included 38 adult women with biopsy-proven BC. Patients underwent a pre-therapy 18F-FDG PET/CT whole-body study and a pre-therapy breast multi-parametric MR study. Patients were then referred for NAC treatment and then for surgical resection, with an evaluation of the therapy response. Segmentation methods were developed in order to identify functional volumes both on 18F-FDG PET images and ADC maps. Macroscopic and histogram features were extracted from the defined functional volumes. Results: Our work demonstrates that macroscopic and histogram features from 18F-FDG PET are able to biologically characterize primitive BC, and define the prognosis. In addition, histogram features from ADC maps are able to predict the response to NAC. Conclusion: Our work suggests that pre-treatment 18F-FDG PET and pre-treatment DWI-MR provide useful complementary information for biological characterization and NAC response prediction in BC.

Purpose Endovascular repair of true visceral artery aneurysms (VAAs) and pseudoaneurysms (VAPAs) with stent-grafting (SG) can simultaneously allow aneurysm exclusion and vessel preservation, minimizing the risk of ischemic complications. Our aim was to report a single-center experience on SG of visceral aneurysms, focusing on technical aspects, clinical outcome and mid-term patency.Materials and Methods Consecutive patients affected by VAAs-VAPAs and submitted to endovascular treatment were retrospectively reviewed, and SG cases with the self-expandable peripheral Viabahn stent-graft were analyzed (2003-2017). Aneurysm type, patient number, SG clinical setting, procedural data, peri-procedural complications, technical success, 30-day clinical success, 30-day mortality and follow-up period (aneurysm exclusion, stent-graft patency, ischemic complications) were analyzed.Results SG was performed in 40 patients (24 VAPAs/16 VAAs) and in 44 procedures (25 in emergency, 19 in elective treatments), via transfemoral in 37 cases (transaxillary in 7 cases). One peri-procedural complication was recorded (a splenic artery dissection successfully converted to transcatheter embolization). The overall technical and clinical success rates were, respectively, 96 and 84%, with excellent trend in elective treatments (both 100%). Overall 30-day mortality was 12.5% (septic shock after pancreatic surgery). Stent-graft thrombosis occurred in 2 patients within 3 months, with aneurysm exclusion and without ischemic complications. Stent-graft patency and aneurysm exclusion were confirmed at 6, 12 and 36 months in 18, 12 and 7 patients, respectively.Conclusion SG of VAAs and VAPAs was safe and effective, particularly in elective treatments. The Viabahn stent-graft, flexible and without shape memory, is suitable for endovascular repair of tortuous visceral arteries.

Background Spinocerebellar ataxias (SCAs) are dominantly inherited, progressive ataxia disorders. Disease progression could be preceded by weight loss. Objectives We aimed to study the course of weight loss in patients who had the most common SCAs (SCA1, SCA2 SCA3, and SCA6). Additional objectives were to identify subgroups of weight evolution, to determine the factors influencing these evolutions, and to assess the impact of these evolutions on disease progression. Methods In total, 384 patients from the EUROSCA prospective cohort study were analyzed who had SCA1, SCA2, SCA3, or SCA6 and at least 3 measurements of weight. Age was used as a time scale. Clinical outcomes were body mass index (BMI) and the Scale for the Assessment and Rating Ataxia (SARA), with scores ranging from 0 to 40. We used a linear mixed model to analyze the course of BMI and a latent class mixed model to identify subgroup BMI evolution. Results Overall, BMI declined over time (−0.11 ± 0.03 kg/m2 per decade; P = 0.0009). Three subgroups of BMI evolution were identified: “decreasing BMI” (n = 88; 23%), “increasing BMI” (n = 70; 18%) and “stable BMI” (n = 226; 59%). Patients in the decreasing BMI group were more severely affected at baseline with higher SARA scores and a higher frequency of non-ataxia signs (especially motor symptoms) compared with those in the other groups. Weight loss was associated with faster disease progression (5.7 ± 0.7 SARA points per decade; P = 0.036). Conclusions The current data have substantial implications for the design of future interventional studies in SCA, as they provide a basis for patient stratification and emphasize the usefulness of BMI as a biomarker for monitoring disease progression.

Visual perceptual learning is widely assumed to reflect plastic changes occurring along the cerebro-cortical visual pathways, including at the earliest stages of processing, though increasing evidence indicates that higher-level brain areas are also involved. Here we addressed the possibility that the cerebellum plays an important role in visual perceptual learning. Within the realm of motor control, the cerebellum supports learning of new skills and recalibration of motor commands when movement execution is consistently perturbed (adaptation). Growing evidence indicates that the cerebellum is also involved in cognition and mediates forms of cognitive learning. Therefore, the obvious question arises whether the cerebellum might play a similar role in learning and adaptation within the perceptual domain. We explored a possible deficit in visual perceptual learning (and adaptation) in patients with cerebellar damage using variants of a novel motion extrapolation, psychophysical paradigm. Compared to their age- and gender-matched controls, patients with focal damage to the posterior (but not the anterior) cerebellum showed strongly diminished learning, in terms of both rate and amount of improvement over time. Consistent with a double-dissociation pattern, patients with focal damage to the anterior cerebellum instead showed more severe clinical motor deficits, indicative of a distinct role of the anterior cerebellum in the motor domain. The collected evidence demonstrates that a pure form of slow-incremental visual perceptual learning is crucially dependent on the intact cerebellum, bearing the notion that the human cerebellum acts as a learning device for motor, cognitive and perceptual functions. We interpret the deficit in terms of an inability to fine-tune predictive models of the incoming flow of visual perceptual input over time. Moreover, our results suggest a strong dissociation between the role of different portions of the cerebellum in motor versusnon-motor functions, with only the posterior lobe being responsible for learning in the perceptual domain. © 2014.

The sense of the body is deeply rooted in humans, and it can be experimentally manipulated by inducing illusions in at least two aspects: a subjective feeling of ownership and a proprioceptive sense of limb position. Previous studies mapped these different aspects onto anatomically distinct neuronal regions, with the ventral premotor cortex processing subjective experience of ownership and the inferior parietal lobule processing proprioceptive calibration. Lines of evidence suggest an involvement also of the cerebellum, but its precise role is not clear yet. To investigate the contribution of the cerebellum in the sense of body ownership, we applied the rubber-hand illusion paradigm in 28 patients affected by neurodegenerative cerebellar ataxia, selectively involving the cerebellum, and in 26 age-matched control participants. The rubber hand illusion is established by synchronous stroking of the participants' real unseen hand and a visible fake hand. Short asynchronous stroking does not bring about the illusion. We tested the subjective experience of the illusion, evaluated through a questionnaire and the proprioceptive drift of the real unseen hand toward the viewed rubber hand. In patients with cerebellar ataxia, we observed reduced sense of the subjective illusory experience specifically after synchronous stroking. In contrast, the proprioceptive drift was enhanced after synchronous and after asynchronous stimulation. These findings support the contention that the mechanisms underlying the presence of the illusion and the proprioceptive drift may be differently affected in different conditions. Impairment of the subjective sense of the illusion in cerebellar patients might hint at an involvement of cerebellar-premotor networks, whereas the proprioceptive drift typically associated with synchronous stroking appears to rely on other circuits, likely involving the cerebellum and the parietal regions.

L’obiettivo della presente ricerca e quello di comprendere le componenti del desiderio sessuale femminile, individuando quei fattori, psicologici, emozionali, situazionali o adattivi, che influenzano l’eccitazione femminile. Sono stati condotti 15 focus group, a cui hanno partecipato 114 donne (eta media = 30 anni, gamma = 18-76 anni). Le partecipanti hanno descritto l’eccitazione sessuale attraverso fattori sia fisiologici (come la lubrificazione vaginale) che emozionali, psicologici e comportamentali. La relazione tra desiderio ed eccitazione e complessa: secondo alcune, il desiderio precede l’eccitazione sessuale, mentre per altre le due fasi avvengono contemporaneamente. Dai risultati emerge una vasta gamma di fattori che inibiscono o aumentano l’eccitazione sessuale, riguardanti se stesse (come l’essere a disagio con il proprio corpo o il provare vergogna), il partner (come la mancanza di complicita o aspetti prettamente fisici del partner) e la situazione. Questi risultati oltre a migliorare la comprensione del desiderio, possono essere utili per la costruzione di questionari che indaghino la sessualita femminile.