Pilot therapeutic education program in multiple system atrophy: Safety, quality of life and satisfaction from a national registry based longitudinal study.

BACKGROUND: Therapeutic education programs are effective in several chronic conditions. However, evidence is lacking in multiple system atrophy (MSA). We aimed to assess efficacy and safety of a comprehensive therapeutic education program in people with MSA (PwMSA) and their caregivers. METHODS: In this prospective longitudinal study we included 16 PwMSA and their main caregivers in 4 groups of 4 dyads each. The program consisted of eight 60-min interdisciplinary sessions: introduction, orthostatic hypotension, speech therapy, gait and respiratory physiotherapy, psychological support, urinary dysfunction, occupational therapy/social work. UMSARS, NMSS, PDQ39, EQ5 and Zarit scales were administered at baseline and 6 months later. After each session participants filled-out a modified EduPark satisfaction questionnaire and a Likert scale. Educational material was generated for each session after suggestions by participants. RESULTS: At baseline PwMSA and caregivers were comparable in age and sex, with significant correlation between UMSARS-IV (disability) and PDQ39 (quality of life). Adherence to sessions was of 94,92 %. Total modified EduPark scores and Likert scales did not differ in PwMSA vs. caregivers, mild-moderate vs. severe-advanced cases or between genders. The significant difference in satisfaction across sessions (p = 0.03) was driven by higher scores in speech, respiratory and occupational therapy sessions. Longitudinally there was no significant worsening in any scale, nor a significant increase post-vs. pre-program in the number of consultations. CONCLUSIONS: The healthcare education program in MSA was feasible, satisfactory, and safe for patients and caregivers. The educational material of the program is being forwarded to incident MSA cases attending our clinic.

Clinical utility of a personalized and long-term monitoring device for Parkinson's disease in a real clinical practice setting: An expert opinion survey on STAT-ON™.

BACKGROUND: STAT-ON™ is an objective tool that registers ON-OFF fluctuations making possible to know the state of the patient at every moment of the day in normal life. Our aim was to analyze the opinion of different Parkinson's disease experts about the STAT-ON™ tool after using the device in a real clinical practice setting (RCPS). METHODS: STAT-ON™ was provided by the Company Sense4Care to Spanish neurologists for using it in a RCPS. Each neurologist had the device for at least three months and could use it in PD patients at his/her own discretion. In February 2020, a survey with 30 questions was sent to all participants. RESULTS: Two thirds of neurologists (53.8% females; mean age 44.9±9 years old) worked in a Movement Disorders Unit, the average experience in PD was 16±6.9 years, and 40.7% of them had previously used other devices. A total of 119 evaluations were performed in 114 patients (range 2-9 by neurologist; mean 4.5±2.3). STAT-ON™ was considered "quite" to "very useful" by 74% of the neurologists with an overall opinion of 6.9±1.7 (0, worst; 10, best). STAT-ON™ was considered better than diaries by 70.3% of neurologists and a useful tool for the identification of patients with advanced PD by 81.5%. Proper identification of freezing of gait episodes and falls were frequent limitations reported. CONCLUSION: STAT-ON™ could be a useful device for using in PD patients in clinical practice.

Combined CSF α-SYN RT-QuIC, CSF NFL and midbrain-pons planimetry in degenerative parkinsonisms: From bedside to bench, and back again.

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.

Clinical utility of a personalized and long-term monitoring device for Parkinson's disease in a real clinical practice setting: An expert opinion survey on STAT-ON™.

BACKGROUND: STAT-ON™ is an objective tool that registers ON-OFF fluctuations making possible to know the state of the patient at every moment of the day in normal life. Our aim was to analyze the opinion of different Parkinson's disease experts about the STAT-ON™ tool after using the device in a real clinical practice setting (RCPS). METHODS: STAT-ON™ was provided by the Company Sense4Care to Spanish neurologists for using it in a RCPS. Each neurologist had the device for at least three months and could use it in PD patients at his/her own discretion. In February 2020, a survey with 30 questions was sent to all participants. RESULTS: Two thirds of neurologists (53.8% females; mean age 44.9±9 years old) worked in a Movement Disorders Unit, the average experience in PD was 16±6.9 years, and 40.7% of them had previously used other devices. A total of 119 evaluations were performed in 114 patients (range 2-9 by neurologist; mean 4.5±2.3). STAT-ON™ was considered "quite" to "very useful" by 74% of the neurologists with an overall opinion of 6.9±1.7 (0, worst; 10, best). STAT-ON™ was considered better than diaries by 70.3% of neurologists and a useful tool for the identification of patients with advanced PD by 81.5%. Proper identification of freezing of gait episodes and falls were frequent limitations reported. CONCLUSION: STAT-ON™ could be a useful device for using in PD patients in clinical practice.

[Facial emotion recognition in children with attention-deficit/hyperactivity disorder]. / Reconocimiento de emociones faciales en niños con trastorno por déficit de atención/hiperactividad.

INTRODUCTION: There are difficulties in relationships that are attributed to a commitment in facial emotions recognition skills in subjects with attention-deficit/hyperactivity disorder (ADHD). The studies carried out did not always take into account the nature of the stimulus selected in pediatrics, which is important because the familiarity in faces, in terms of their physiognomic characteristics, makes it easier the recognition of the expressions. AIM: To identify the profile of recognition of emotions in pediatric subjects with ADHD, using as a stimulus photos of boys and girls. SUBJECTS AND METHODS: Analytical cross-sectional study of cases and controls. We analyzed the results obtained from 54 participants between 7 and 13 years divided into two groups: 26 in control group and 28 in ADHD group. They conducted an assessment with the Wechsler Intelligence Scale for Children (WISC-V), Conners' Continuous Performance Test II (CPT-II), and Child Affective Facial Expression set (CAFE). RESULTS: The ADHD group showed a lower performance in recognition of emotions in general, with greater difficulty in the recognition of anger, surprise and neutral expression in particular. No relationship was found between the recognition of emotions and age, sex, total intelligence quotient or CPT-II care variables. CONCLUSIONS: The subjects with ADHD present deficits in the recognition of emotions, especially in the recognition of anger, surprise and neutral expression, which could explain the difficulties in the interaction and that should be treated within the therapeutic framework.

TITLE: Reconocimiento de emociones faciales en niños con trastorno por déficit de atención/hiperactividad.Introducción. En sujetos con trastorno por déficit de atención/hiperactividad (TDAH), se observan dificultades en las relaciones interpersonales que se atribuyen a una afectación en las habilidades de reconocimiento de emociones faciales. Los estudios realizados no siempre tuvieron presente la naturaleza del estímulo seleccionado en pediatría, lo cual es fundamental porque la familiaridad en los rostros, en cuanto a sus características fisonómicas, favorece el reconocimiento de las expresiones. Objetivo. Identificar el perfil de reconocimiento de emociones en sujetos pediátricos con TDAH, utilizando como estímulo fotos de niños y niñas. Sujetos y métodos. Estudio analítico de corte transversal de casos y controles. Se analizaron los resultados obtenidos de 54 participantes de 7-13 años divididos en dos grupos: 26 en el grupo control y 28 en el grupo con TDAH. Realizaron una evaluación con la escala de inteligencia de Wechsler para niños (WISC-V), el Conners' Continuous Performance Test II (CPT-II) y el Child Affective Facial Expression set (CAFE). Resultados. El grupo con TDAH mostró un rendimiento menor en el reconocimiento de emociones en general, con mayor dificultad en el reconocimiento de enojo, sorpresa y expresión neutral en particular. No se encontró una relación entre el reconocimiento de emociones y la edad, el sexo, el cociente intelectual total o las variables de atención del CPT-II. Conclusiones. Los sujetos con TDAH presentan déficits en el reconocimiento de emociones, especialmente en el reconocimiento de enojo, sorpresa y expresión neutral, que podrían explicar las dificultades en la interacción y deberían tratarse dentro del marco terapéutico.

Early treatment of Class III malocclusion with RME and facial mask: evaluation of dentoalveolar effects on digital dental casts.

AIM: To analyse the dental effects on digital dental casts in subjects with Class III malocclusion treated with bonded Rapid Maxillary Expansion and Facial Mask (RME/FM). MATERIALS AND METHODS: A study group sample (SG) of 29 subjects (13 females and 16 males) and mean age of 7.4 years (SD 1.2 years) was selected. A bonded RME was placed and activated 1/4 of a turn per day until overcorrection of the transverse width. At the end of expansion, patients were given FMs and treated at least to a positive overjet. The SG was compared with a control group (CG) of 21 prepubertal subjects (9 females; 12 males) presenting with normal occlusion and mean age of 7.9 years (SD 1.6 years). For each subject of the SG and CG initial (pretreatment, T1) and final (post-treatment, T2) digital dental casts were available. Significant in between-group differences were tested with the Student's t-test. RESULTS: The transverse dimension of the upper arch was significantly greater in SG vs. CG (IMAW: +2.6 mm; ICAW: +3.1 mm). Anterior arch length and arch depth were significantly smaller in SG when compared with CG (AAL: -3.2 mm, AD: -3.4 mm) at the end of therapy. CONCLUSION: The orthopaedic treatment with bonded RME/FM produced in subjects treated in the deciduous or early mixed dentition a significant expansion of the maxillary arch and mesialisation of the posterior teeth with a reduction of the arch depth.

123I-MIBG cardiac uptake, smell identification and 123I-FP-CIT SPECT in the differential diagnosis between vascular parkinsonism and Parkinson's disease.

UNLABELLED: Vascular parkinsonism (VP) may occur as a distinct clinicopathological entity but the comorbid presence of vascular damage in Parkinson's disease (PD) is very frequent too. This differential diagnosis has therapeutic and prognostic implications but remains challenging as the usefulness of a number of supporting tools is still controversial. OBJECTIVE: To ascertain the clinical value of cardiac (123)I-meta-iodobenzylguanidine ((123)I-MIBG) SPECT, olfactory function and (123)I-FP-CIT SPECT as supporting tools in the differential diagnosis between VP and PD. METHODS: Cross-sectional study of 15 consecutive patients with suspected VP, 15 PD patients and 9 healthy subjects. Cardiac (123)I-MIBG SPECT (heart-to-mediastinum ratio) and olfactory testing (University of Pennsylvania Smell Identification Test-UPSIT) were performed in all of them. (123)I-FP-CIT SPECT was performed in VP-suspected patients. RESULTS: Heart-to-mediatinum ratio was significant lower in suspected VP (mean 1.45) and PD (mean 1.16) compared to control group (mean 1.69) (p = 0.017 and p < 0.0001). VP patients presented a higher ratio than PD patients (p = 0.001). Control group presented a significant higher UPSIT score (mean 30.71) when compared to both VP (mean 18.33) and PD (mean 15.29) (p = 0.001 for both groups). Those VP with a cardiac (123)I-MIBG non suggestive of PD were more likely to have a higher UPSIT score (p = 0.006). (123)I-FP-CIT SPECT imaging was heterogeneous (7/15 VP normal, 3/15 abnormal suggestive of PD and 5/15 abnormal but atypical for PD). CONCLUSIONS: The use of cardiac (123)I-MIBG SPECT and to a lesser extent UPSIT could assist the differential diagnosis between VP and PD in subjects in which the diagnosis remains uncertain despite (123)I-FP-CIT SPECT imaging.

Optimization of systemic treatments for chronic plaque psoriasis. Recommendations for switching and transitioning.

AIM: The aim of this study was to provide practical recommendations for optimizing the use of conventional and biological systemic treatments for moderate-severe chronic plaque psoriasis, particularly in case of transitioning and switching. METHODS: A total number of 147 dermatologists from 33 different countries including Italy achieved consensus in providing practical recommendations for the use of conventional and biological treatments for moderate to severe psoriasis based on systematic literature review and/or expert opinion. RESULTS: In general, the continuous treatment regimen should be preferred in order to achieve a complete and long-term control of psoriasis. However, the treatment could be stopped or the dose reduced in case of complete disease clearance. A conventional drug could be associated to biological treatment in selected cases. Transitioning and/or switching could be considered in case of inefficacy or intolerance. A period of wash up is required if transitioning or switching is due to safety issues. CONCLUSION: This study provides practical suggestions for the optimal use of conventional and biological treatments for chronic plaque psoriasis.

Socio-demographic and clinical factors influencing the adherence to treatment in Parkinson's disease: the ADHESON study.

BACKGROUND: Symptoms of Parkinson's disease (PD) are usually controlled by a continuous titration of medication and addition of multiple therapies over the course of the disease. Therapeutic complex schemes, polymedication, comorbidities and the number of medications required contribute to non-adherence. METHODS: This cross-sectional survey was performed in 418 patients with PD on treatment with any antiparkinsonian medication. Patient adherence was assessed through physicians' subjective perception and the Morisky-Green test (MGT). Several social, demographic and clinical features were correlated through bivariate and multivariate analyses. RESULTS: According to the physician's opinion 93.7%, and according to the MGT 60.4% of patients were adherent to parkinsonian therapy. The bivariate analysis showed greater adherence in patients with a high level of knowledge about the disease (62.8%), good clinical control (63.6%), a spouse or life partner (63%) and higher incomes (66%). Negative correlation with psychiatric symptoms was found. In relation to the MGT, the logistic regression model showed a negative correlation between cognitive deterioration and psychiatric pathology and adherence to therapy. CONCLUSIONS: The physician's impression overestimated the compliance of patients when compared with an objective evaluation such as the MGT. Cognitive impairment and psychiatric symptoms are the clinical variables associated with a lower level of adherence.

Non invasive evaluation of endothelial function in patients with Anderson-Fabry disease.

AIM: Fabry's disease is an X-linked recessive abnormality of glycosphingolipid metabolism. Increased levels of endothelial prothrombotic factors have recently been demonstrated in Fabry's disease, whereas endothelial function has not been studied using high resolution ultrasound. METHODS: We enrolled 6 patients (4 male, 2 female; mean age, 37 years) and 12 sex matched control subjects (mean age, 37 years). Patients' exclusion criteria included a prior history of cardiac disease, diabetes and treated or untreated hypertension. Patients underwent: anamnesis, physical examination, EKG, 2-dimensional echocardiography with tissue Doppler, measurement of body weight and height, blood pressure. Biochemistry variables were also considered: fasting blood sugar, total cholesterol, HDL-C, LDL-C, triglycerides, fibrinogen, C reactive protein and homocysteine. Using high resolution ultrasound, we assessed the brachial vasodilator response to reactive hyperemia (endothelium-dependent) and sublingual nitroglycerin (NTG) (endothelium-independent). Flow-mediated dilatation (FMD) was expressed as percentage change in post-stimulus diameter in comparison with the baseline. RESULTS: In baseline condition, there was no significant difference between patients and controls in the brachial artery diameter (3.5+/-0.55 vs 3.1+/-0.4). After reactive hyperemia, the FMD change was significantly higher in controls than in patients (16.5+/-6.3% vs 9.3+/-6.2%, P<0.05). After NTG, endothelium-independent vasodilation did not show a significant difference between cases and controls (15+/-7.7% vs 13.8+/-7.1%). CONCLUSIONS: Our study demonstrated the presence of endothelial dysfunction in patients with Fabry's disease in comparison to controls. We hypothesized that endothelial dysfunction may contribute to the pathogenesis of ischemic events in patients with Fabry's disease.