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.

Síndrome de Angelman en el adulto / Angelman syndrome in adulthood

Introducción: El síndrome de Angelman (SA) está ampliamente descrito en la infancia, pero existen escasos estudios en edad adulta y la mayoría recoge un pequeño número de pacientes o condiciones específicas, como epilepsia o sueño. Objetivo: El objetivo de este estudio es describir el SA en la edad adulta en nuestro centro, sus necesidades especiales, y el soporte médico y social para mejorar la atención y ofrecer una mejor transición del servicio de pediatría a las unidades de adultos. Pacientes y métodos: Se recogen pacientes con SA genéticamente confirmado, y describimos datos demográficos, médicos y sociales mediante la revisión de historias clínicas, entrevistas telefónicas con el cuidador principal y tres escalas estandarizadas de sueño, dependencia y calidad de vida. Resultados: Se incluye a 30 pacientes con una mediana de edad de 22,7 años: 22 son deleciones, 27 presentan antecedente de epilepsia y 13 están en tratamiento con, al menos, dos fármacos antiepilépticos. Las comorbilidades más frecuentes después de la epilepsia fueron los síntomas psiquiátricos, la escoliosis, el sobrepeso, el estreñimiento y problemas oftalmológicos. El 40% precisó ingresos hospitalarios en la edad adulta, cinco están institucionalizados y 24 reciben terapias no médicas. El médico a cargo es el neurólogo en la mayoría, seguido del neuropediatra. Conclusiones: Es necesario realizar estudios de historia natural más allá de la infancia. Ésta es la primera revisión española de adultos con SA que recoge un amplio espectro de condiciones sociales y médicas de estos pacientes.(AU)

Introduction: Angelman syndrome (AS) is widely described in childhood, but few studies have been conducted in adulthood and most of them report a small number of patients or specific conditions, such as epilepsy or sleep. Aim: The aim of this study is to describe AS in adulthood in our centre, the special needs it requires, and the medical and social support to improve care and to provide a better transition from the paediatric service to units for adults. Patients and methods: We collected patients with genetically confirmed AS, and described demographic, medical and social data by reviewing medical records, telephone interviews with the primary caregiver and three standardised sleep, dependency and quality of life scales. Results: Thirty patients with a median age of 22.7 years were included: 22 were deletions, 27 had a history of epilepsy and 13 were on treatment involving at least two antiepileptic drugs. The most frequent comorbidities after epilepsy were psychiatric symptoms, scoliosis, overweight, constipation and ophthalmological problems. Forty per cent required hospital admissions in adulthood, five were institutionalised and 24 received non-medical therapies. The doctor in charge was the neurologist in most cases, followed by the neuropaediatrician. Conclusions: Studies that examine the natural history beyond childhood are warranted. This is the first Spanish review of adults with AS that covers a broad spectrum of social and medical conditions of these patients.(AU)

[Angelman syndrome in adulthood]. / Síndrome de Angelman en el adulto.

INTRODUCTION: Angelman syndrome (AS) is widely described in childhood, but few studies have been conducted in adulthood and most of them report a small number of patients or specific conditions, such as epilepsy or sleep. AIM: The aim of this study is to describe AS in adulthood in our centre, the special needs it requires, and the medical and social support to improve care and to provide a better transition from the paediatric service to units for adults. PATIENTS AND METHODS: We collected patients with genetically confirmed AS, and described demographic, medical and social data by reviewing medical records, telephone interviews with the primary caregiver and three standardised sleep, dependency and quality of life scales. RESULTS: Thirty patients with a median age of 22.7 years were included: 22 were deletions, 27 had a history of epilepsy and 13 were on treatment involving at least two antiepileptic drugs. The most frequent comorbidities after epilepsy were psychiatric symptoms, scoliosis, overweight, constipation and ophthalmological problems. Forty per cent required hospital admissions in adulthood, five were institutionalised and 24 received non-medical therapies. The doctor in charge was the neurologist in most cases, followed by the neuropaediatrician. CONCLUSIONS: Studies that examine the natural history beyond childhood are warranted. This is the first Spanish review of adults with AS that covers a broad spectrum of social and medical conditions of these patients.

TITLE: Síndrome de Angelman en el adulto.Introducción. El síndrome de Angelman (SA) está ampliamente descrito en la infancia, pero existen escasos estudios en edad adulta y la mayoría recoge un pequeño número de pacientes o condiciones específicas, como epilepsia o sueño. Objetivo. El objetivo de este estudio es describir el SA en la edad adulta en nuestro centro, sus necesidades especiales, y el soporte médico y social para mejorar la atención y ofrecer una mejor transición del servicio de pediatría a las unidades de adultos. Pacientes y métodos. Se recogen pacientes con SA genéticamente confirmado, y describimos datos demográficos, médicos y sociales mediante la revisión de historias clínicas, entrevistas telefónicas con el cuidador principal y tres escalas estandarizadas de sueño, dependencia y calidad de vida. Resultados. Se incluye a 30 pacientes con una mediana de edad de 22,7 años: 22 son deleciones, 27 presentan antecedente de epilepsia y 13 están en tratamiento con, al menos, dos fármacos antiepilépticos. Las comorbilidades más frecuentes después de la epilepsia fueron los síntomas psiquiátricos, la escoliosis, el sobrepeso, el estreñimiento y problemas oftalmológicos. El 40% precisó ingresos hospitalarios en la edad adulta, cinco están institucionalizados y 24 reciben terapias no médicas. El médico a cargo es el neurólogo en la mayoría, seguido del neuropediatra. Conclusiones. Es necesario realizar estudios de historia natural más allá de la infancia. Ésta es la primera revisión española de adultos con SA que recoge un amplio espectro de condiciones sociales y médicas de estos pacientes.

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.

Non-motor symptom burden in patients with Parkinson's disease with impulse control disorders and compulsive behaviours: results from the COPPADIS cohort.

The study was aimed at analysing the frequency of impulse control disorders (ICDs) and compulsive behaviours (CBs) in patients with Parkinson's disease (PD) and in control subjects (CS) as well as the relationship between ICDs/CBs and motor, nonmotor features and dopaminergic treatment in PD patients. Data came from COPPADIS-2015, an observational, descriptive, nationwide (Spain) study. We used the validated Questionnaire for Impulsive-Compulsive Disorders in Parkinson's Disease-Rating Scale (QUIP-RS) for ICD/CB screening. The association between demographic data and ICDs/CBs was analyzed in both groups. In PD, this relationship was evaluated using clinical features and treatment-related data. As result, 613 PD patients (mean age 62.47 ± 9.09 years, 59.87% men) and 179 CS (mean age 60.84 ± 8.33 years, 47.48% men) were included. ICDs and CBs were more frequent in PD (ICDs 12.7% vs. 1.6%, p < 0.001; CBs 7.18% vs. 1.67%, p = 0.01). PD patients had more frequent previous ICDs history, premorbid impulsive personality and antidepressant treatment (p < 0.05) compared with CS. In PD, patients with ICDs/CBs presented younger age at disease onset, more frequent history of previous ICDs and premorbid personality (p < 0.05), as well as higher comorbidity with nonmotor symptoms, including depression and poor quality of life. Treatment with dopamine agonists increased the risk of ICDs/CBs, being dose dependent (p < 0.05). As conclusions, ICDs and CBs were more frequent in patients with PD than in CS. More nonmotor symptoms were present in patients with PD who had ICDs/CBs compared with those without. Dopamine agonists have a prominent effect on ICDs/CBs, which could be influenced by dose.

Maternal caesarean section infection (MACSI) in Sierra Leone: a case-control study.

Sierra Leone is the country with highest maternal mortality and infections are the underlying cause in 11% of maternal deaths, but the real burden remains unknown. This study aims to determine the incidence and risk factors of surgical site infection (SSI) post-caesarean section (CS) in women admitted to Princess Christian Maternity Hospital (PCMH) in Freetown, Sierra Leone. A prospective case-control (1:3 ratio) study was implemented from 1 May 2018 to 30 April 2019 and 11 women presenting with suspected or confirmed infection post-CS were screened for inclusion as a case. For each case, three patients undergoing CS on the same day and admitted to the same ward, but not presenting with SSI, were selected as controls. The post-CS infection rate was 10.9%. Two hundred and fifty-four clinically confirmed cases were enrolled and matched with 762 control patients. By multivariable analysis, the risk factors for SSI were: being single (odds ratio (OR) 1.48, 95% confidence interval (CI) 1.36-1.66), low education level (OR 1.68, 95% CI 1.55-1.84), previous CS (OR 1.27, 95% CI 1.10-1.52), presenting with premature membranes rupture (OR 1.49, 95% CI 1.18-1.88), a long decision-incision time (OR 2.08, 95% CI 1.74-2.24) and a high missing post-CS antibiotic doses rate (OR 2.52, 95% CI 2.10-2.85).

One Health and antimicrobial resistance, a United States perspective.

Antimicrobial drugs are a precious resource, responsible for saving millions of lives since their discovery. Unfortunately, some antimicrobials are rapidly losing their effectiveness due to the development and spread of antimicrobial resistance (AMR), a multi-faceted and complex problem affecting humans, animals, plants and the environment. While AMR is a global problem, in this paper, the authors briefly highlight some ongoing efforts in the United States of America aimed at integrating a One Health approach into policies and programmes that address this important health threat.

Les antibiotiques sont des ressources de grande valeur qui ont sauvé des millions de vies depuis leur découverte. Malheureusement, certains agents antimicrobiens perdent rapidement leur efficacité en raison de l'apparition et propagation des résistances à ces agents, phénomène complexe et multidimensionnel qui affecte l'homme, les animaux, les plantes et l'environnement. La résistance aux agents antimicrobiens est un problème mondial ; dans cet article, les auteurs décrivent certaines initiatives actuellement mises en oeuvre aux États-Unis d'Amérique pour intégrer l'approche Une seule santé dans les politiques et les programmes conçus pour lutter contre cette menace sanitaire majeure.

Los fármacos antimicrobianos son un recurso valiosísimo, cuyo uso ha salvado millones de vidas desde que fueron descubiertos. Lamentablemente, algunos de ellos están perdiendo rápidamente eficacia debido a la aparición y propagación de resistencias, lo que plantea un problema tan complejo como poliédrico, que afecta a personas, animales, plantas y ecosistemas. Aunque la dimensión del problema es planetaria, los autores destacan aquí brevemente algunas de las iniciativas en curso en los Estados Unidos de América que tienen por objetivo integrar los planteamientos de Una sola salud en el conjunto de políticas y programas desde los cuales se aborda esta importante amenaza sanitaria.

Monitoring game-based motor rehabilitation of patients at home for better plans of care and quality of life.

This paper describes the biomedical, remote monitoring infrastructure developed and currently tested in the EU REHAB@HOME project to support home rehabilitation of the upper extremity of persons post-stroke and in persons with other neurological disorders, such as Multiple Sclerosis patients, in order to track their progress over therapy and improve their Quality of Life. The paper will specifically focus on describing the initial testing of the tele-rehabilitation system's components for patients' biomedical monitoring over therapy, which support the delivery and monitoring of more personalized, engaging plans of care by rehabilitation centers and services.

The Actis-gouge: a simple cutting tool for proper muscular resection in hypertrophic cardiomyopathy.

BACKGROUND: Surgical treatment of hypertrophic cardiomyopathy (HC) may be challenging for the risk of surgical complications or insufficient resection. We present our cutting tool to perform proper muscular resection in HC. MATERIAL AND METHODS: Ten patients (5 males, mean age 43,1 +/- 19,6 years, range 9-70 years) were operated on for HC using this semicircular cutting device. Combined procedures were : mitral valve repair (n = 1), mitral valve replacement (n = 2), right ventricular myectomy (n = 1), aortic valve replacement (n = 1), mitral and aortic replacement (n = 1). RESULTS: There was one early death. All the surviving patients are alive over a variable follow up from 2 to 8 years, with consistent reduction of symptoms: in fact, no patient had residual angina with significant reduction of the NYHA class from 3,2 +/- 0,6 to 1,3 +/- 0,5 postoperatively (p < 0,05). Muscular resection was effective with significant reduction of sub-valvular gradient from 84.5 + 33,4 mmHg to 14,1 +/- 17,6 mmHg (p < 0,05) without complications such as complete atrio-ventricular block or ventricular septal defects. CONCLUSION: Our semicircular myotome is an effective tool to perform a safe myectomy and it avoids surgical complications such as atrio-ventricular blocks or sub-valvular injuries. Our experience suggests that this cutting tool offers a reproducible method for muscular resection and it shows appreciable effects in the reduction of sub-valvular gradient with promising results in terms of morbidity and mortality.

Transmyocardial laser revascularization. Personal experience.

BACKGROUND: Indirect revascularization is a therapeutic approach in case of severe angina not suitable for percutaneous or surgical revascularization. Transmyocardial revascularization (TMR) is one of the techniques used for indirect revascularization and it allows to create transmyocardial channels by a laser energy bundle delivered on left ventricular epicardial surface. Benefits of the procedure are related mainly to the angiogenesis caused by inflammation and secondly to the destruction of the nervous fibers of the heart. PATIENTS AND METHOD: From September 1996 up to July 1997, 14 patients (9 males - 66.7%, mean age 64.8±7.9 years) underwent TMR. All patients referred angina at rest; Canadian Angina Class was IV in 7 patients (58.3%), III in 5 (41.7%). Before the enrollment, coronarography was routinely performed to find out the feasibility of Coronary Artery Bypass Graft (CABG): 13 patients (91,6%) had coronary arteries lesions not suitable for direct revascularization; this condition was limited only to postero-lateral area in one patient submitted to combined TMR + CABG procedures. RESULTS: Mean discharge time was 3,2±1,3 days after surgery. All patients were discharged in good clinical conditions. Perfusion thallium scintigraphy was performed in 7 patients at a mean follow-up of 4±2 months, showing in all but one an improvement of perfusion defects. Moreover an exercise treadmill improvement was observed in the same patients and all of them are in good clinical conditions, with significantly reduced use of active drugs. CONCLUSION; Our experience confirms that TMR is a safe and feasible procedure and it offers a therapeutic solution in case of untreatable angina. Moreover, it could be a hybrid approach for patients undergoing CABGs in case of absence of vessels suitable for surgical approach in limited areas of the heart.

Rolling and slipping motion of Euler's disk.

We present an experimental study of the motion of a circular disk spun onto a table. With the help of a high speed video system, the temporal evolution of (i) the inclination angle alpha, (ii) the angular velocity omega, and (iii) the precession rate Omega are studied. The influence of the mass of the disk as well as the friction between the disk and the supporting surface are considered. Both inclination angle and angular velocity are observed to decrease according to a power law. We also show that the precession rate diverges as the motion stops. Measurements are performed very near the collapse as well as on long range times. Times to collapse have been also measured. Results are compared with previous theoretical and experimental works. The major source of energy dissipation is found to be the slipping of the disk on the plane.

[Serum levels of vitamin C in young adults who chronically use drugs of abuse]. / Niveles séricos de vitamina C en adultos jóvenes consumidores crónicos de drogas de abuso.

Few studies have evaluated the relationship between drugs of abuse consumption and plasma levels of vitamin C. Because of the importance of vitamin C due to its role in prevention of acute and chronic diseases, this study was carried out with the purpose of testing the influence of consumption of drugs of abuse on the plasmatic levels of vitamin C (ascorbic acid) of 56 male chronic users of drugs of abuse with an age range of 16 to 40 years. The following was performed: 1) A survey of consumption to determine the kind, frequency and quantity of drug(s) used and 2) The plasmatic levels of vitamin C in fasting condition, using the Rue and Kuether method. The results obtained showed that 89% of the population under study used drugs for the first time before the age of 18, and 78.4% started with marijuana; 60.7% of them were mixed drug users. The average level of plasmatic ascorbic acid was 0.89 +/- 0.06 mg/dL, nevertheless, according to nutritional category, 76% have values greater than 0.4 mg/dL and 23.2% were at moderated risk (0.35 +/- 0.01 mg/dL) of vitamin C deficiency. Likewise, classifying them in the antioxidant category (according to the suggested values of Gey, 1993) it was obtained that 55.4% had suboptimal or inadequate concentrations to carry out its antioxidant protective function. The length of time of drug's consumption influenced on the ascorbic acid level too. It can be concluded that chronic consumption of drugs of abuse can negatively influence the plasmatic levels of ascorbic acid, leading these patients to a latent condition of vitamin C deficiency. Finally it is necessary to deepen the study of vitamin C levels of users of drugs of abuse.

A simple method to correct aortic tube graft kinking without cardiopulmonary by-pass and aortic clamping.

A method for correcting the tube kinking after ascending aortic replacement for acute dissection is described. Its main advantage is the no need for cardiopulmonary by-pass (CPB) and aortic clamping to solve the problem.

Aortobronchial fistula after aortic dissection type B.

Although rare, aortobronchial fistula complicates thoracic aortic surgery. Correct diagnosis and the infectious nature of the lesion are the most important conditions to define, for the following best therapy. We presented a case of non-infectious postsurgical aortobronchial fistula, revealed by computed-tomographic scan and angiography procedure, treated with prosthetic graft replacement and broad spectrum antibiotic therapy. In the case of infection our policy is homograft replacement. Computed tomography, being able to make diagnosis, should be performed as the initial technique.