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.

Palliative care management in patients with Parkinson's disease and other movement disorders in Spain. National survey of neurologists.

INTRODUCTION: Palliative care in neurodegenerative diseases is useful but underused. The objective of this study is to know how palliative care (PC) is applied in Spain in order to identify limitations and unmet needs. MATERIALS AND METHODS: It is a descriptive, observational, cross-sectional study, anonymous survey type of 20 questions, directed and answered by neurologists dedicated to movement disorders (MD) in Spain. RESULTS: 58 responses were obtained from neurologists from 15 autonomous communities. 69% answered that they did not have a specialised MD nursing facility but did have a PC team in their centre (81%). No specific protocol for PC in MD was identified. All except one neurologist stated that they lacked sufficient training in PC, the main training need being the "advance directives explanation". Only 1 in 4 neurologists answered routinely explaining advance healthcare planning to their patients, recognising up to 84.5% of neurologists not knowing how to assess the patient's competence. 60.3% of those surveyed answered that between 10% and 30% of their patients would be candidates for PC, although 1 in 3 said they were not clear when to refer the patient to PC. 100% of neurologists affirmed the priority need to implement PC protocols in MD. CONCLUSIONS: Our study shows a formative deficit in PC in this area and in the care of the patient with movement disorders and their environment, and should serve as a starting point to develop consensual care protocols.

Autosomal recessive spinocerebellar ataxia SCAR8/ARCA1: first families detected in Spain.

INTRODUCTION: Autosomal recessive spinocerebellar ataxia type 8 (ARCA1/SCAR8) is caused by mutations of the SYNE1 gene. The disease was initially described in families from Quebec (Canada) with a phenotype of pure cerebellar syndrome, but in recent years has been reported with a more variable clinical phenotype in other countries. Cases have recently been described of muscular dystrophy, arthrogryposis, and cardiomyopathy due to SYNE1 mutations. OBJECTIVE: To describe clinical and molecular findings from 4 patients (3 men and one woman) diagnosed with ARCA1/SCAR8 from 3 Spanish families from different regions. MATERIAL AND METHODS: We describe the clinical, paraclinical, and genetic results from 4 patients diagnosed with ARCA1/SCAR8 at different Spanish neurology departments. RESULTS: Onset occurred in the third or fourth decade of life in all patients. After 15 years of progression, 3 patients presented pure cerebellar syndrome, similar to the Canadian patients; the fourth patient, with over 30 years' progression, presented vertical gaze palsy, pyramidal signs, and moderate cognitive impairment. In all patients, MRI studies showed cerebellar atrophy. The genetic study revealed distinct pathogenic SYNE1 mutations in each family. CONCLUSIONS: ARCA1/SCAR8 can be found worldwide and may be caused by many distinct mutations in the SYNE1 gene. The disease may manifest with a complex phenotype of varying severity.

Validation of the Spanish-language version of the Montreal Cognitive Assessment as a screening test for cognitive impairment in multiple sclerosis.

BACKGROUND: The neuropsychological batteries traditionally used for the assessment of cognitive impairment (CI) in patients with multiple sclerosis are complex tests requiring a long time to administer. Simpler tests are needed to detect cognitive impairment in daily clinical practice. OBJECTIVE: We aimed to evaluate the diagnostic validity and reliability of the Montreal Cognitive Assessment (MoCA) test as a screening tool for CI in patients with multiple sclerosis, as compared against the Brief Neuropsychological Battery. MATERIAL AND METHODS: We recruited 52 patients with multiple sclerosis (61.5% women; mean age [standard deviation]: 41.7 [11.5] years). We analysed the reliability (internal consistency, interobserver reliability, and test-retest reliability), construct validity (factor analysis, Pearson correlation coefficient, and coefficient of determination), and criterion validity (ROC curve, sensitivity, specificity, total agreement, positive and negative predictive values, positive and negative likelihood ratios, and Fagan nomogram) of the MoCA test in this population. RESULTS: The prevalence of CI was 21.2% according to findings from the Brief Neuropsychological Battery, and 25% according to the MoCA test. The MoCA test showed good internal consistency (Cronbach alpha, 0.822) and interobserver and test-retest reliability (intraclass correlation coefficient 0.80 and 0.96, respectively). The correlation coefficient between total Brief Neuropsychological Battery and MoCA test scores was 0.82. The optimal cut-off point on the ROC curve was 25-26, yielding 91% sensitivity and 93% specificity. CONCLUSION: The MoCA test is a valid and reliable tool for screening for CI in patients with multiple sclerosis.

Management of Parkinson's disease and other movement disorders in woman of childbearing age: Part 1. / Manejo de la enfermedad de Parkinson y otros trastornos del movimiento en mujeres en edad fértil: Parte 1.

INTRODUCTION: The main challenge of Parkinson's disease in women of childbearing age is managing symptoms and drugs during pregnancy and breastfeeding. The increase in the age at which women are having children makes it likely that these pregnancies will become more common in future. OBJECTIVES: This study aims to define the clinical characteristics of women of childbearing age with Parkinson's disease and the factors affecting their lives, and to establish a series of guidelines for managing pregnancy in these patients. RESULTS: This consensus document was developed through an exhaustive literature search and a discussion of the available evidence by a group of movement disorder experts from the Spanish Society of Neurology. CONCLUSIONS: Parkinson's disease affects all aspects of sexual and reproductive health in women of childbearing age. Pregnancy should be well planned to minimise teratogenic risk. A multidisciplinary approach should be adopted in the management of these patients in order to take all relevant considerations into account.

Management of Parkinson's disease and other movement disorders in women of childbearing age: Part 2. / Manejo de la enfermedad de Parkinson y otros trastornos del movimiento en mujeres en edad fértil: parte 2.

INTRODUCTION: Many diseases associated with hyperkinetic movement disorders manifest in women of childbearing age. It is important to understand the risks of these diseases during pregnancy, and the potential risks of treatment for the fetus. OBJECTIVES: This study aims to define the clinical characteristics and the factors affecting the lives of women of childbearing age with dystonia, chorea, Tourette syndrome, tremor, and restless legs syndrome, and to establish guidelines for management of pregnancy and breastfeeding in these patients. RESULTS: This consensus document was developed through an exhaustive literature search and a discussion of the content by a group of movement disorder experts from the Spanish Society of Neurology. CONCLUSIONS: We must evaluate the risks and benefits of treatment in all women with hyperkinetic movement disorders, whether pre-existing or with onset during pregnancy, and aim to reduce effective doses as much as possible or to administer drugs only when necessary. In hereditary diseases, families should be offered genetic counselling. It is important to recognise movement disorders triggered during pregnancy, such as certain types of chorea and restless legs syndrome.

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.

[Clinical experience in the treatment of motor fluctuations in Parkinson's disease. Delphi consensus of a group of experts in movement disorders]. / Experiencia clínica en el tratamiento de las fluctuaciones motoras en la enfermedad de Parkinson. Consenso Delphi de un grupo de expertos en trastornos del movimiento.

INTRODUCTION: Motor fluctuations are one of the most common complications of Parkinson's disease and their treatment is still a complex matter. Therefore, from the Neurology Movement Disorders Group we present our clinical experience in the treatment of these complications, with the intention of it being useful in decision-making in daily clinical practice. DEVELOPMENT: Nineteen questions were developed based on a literature review and an open survey answered by members of this group. These issues were discussed in two phases, using the Delphi methodology. Considering the results of the survey, levodopa dose adjustment and dopamine agonists are the option with the best efficacy/tolerability ratio in the treatment of motor fluctuations. Rotigotine is useful in the motor fluctuations associated with gastroparesis, and intermittent subcutaneous apomorphine has positive effects in patients with unpredictable off periods. The most relevant adverse effect associated with dopamine agonists is impulse control disorder. Catechol-O-methyltransferase inhibitors are useful in the initial stages of motor fluctuations, especially in wearing off. Monoamine oxidase inhibitors are generally drugs that are well-tolerated and useful in motor fluctuations. If these measures are not effective, second-line treatments should be indicated on a case-by-case basis. CONCLUSION: The clinical profile of patients with Parkinson's disease is paramount in deciding the most appropriate therapy for the treatment of motor fluctuations.

TITLE: Experiencia clínica en el tratamiento de las fluctuaciones motoras en la enfermedad de Parkinson. Consenso Delphi de un grupo de expertos en trastornos del movimiento.Introducción. Las fluctuaciones motoras son una de las complicaciones más frecuentes en la enfermedad de Parkinson y su tratamiento sigue siendo complejo. Por ello, desde el Grupo de Trastornos del Movimiento de la Asociación Madrileña de Neurología presentamos nuestra experiencia clínica en el tratamiento de estas complicaciones, con la intención de que sea de utilidad en la toma de decisiones en la práctica clínica diaria. Desarrollo. Se elaboraron 19 preguntas a partir de una revisión bibliográfica y una encuesta abierta respondida por los miembros de dicho grupo. Dichas cuestiones se debatieron en dos fases, utilizando la metodología Delphi. Considerando los resultados de la encuesta, el ajuste de la dosis de levodopa y los agonistas dopaminérgicos son la opción con mejor relación eficacia/tolerabilidad en el tratamiento de las fluctuaciones motoras. La rotigotina es útil en las fluctuaciones motoras asociadas a gastroparesia, y la apomorfina subcutánea intermitente, en pacientes con off impredecible. El efecto adverso más relevante asociado a los agonistas dopaminérgicos es el trastorno del control de impulsos. Los inhibidores de la catecol-O-metiltransferasa son útiles en las fluctuaciones motoras de inicio, especialmente en el wearing off. Los inhibidores de la monoaminooxidasa son fármacos, en general, bien tolerados y útiles en las fluctuaciones motoras. En caso de que estas medidas no resulten eficaces, se deben indicar terapias de segunda línea de manera individualizada. Conclusión. El perfil clínico del paciente con enfermedad de Parkinson es primordial para decidir la terapia más adecuada en el tratamiento de las fluctuaciones motoras.

Validation of the Spanish-language version of the Montreal Cognitive Assessment as a screening test for cognitive impairment in multiple sclerosis. / Validación de la versión española de la Escala Cognitiva de Montreal (MoCA) como herramienta de cribado de deterioro cognitivo asociado a la esclerosis múltiple.

INTRODUCTION: The neuropsychological batteries traditionally used for the assessment of cognitive impairment (CI) in patients with multiple sclerosis are complex tests requiring a long time to administer. Simpler tests are needed to detect cognitive impairment in daily clinical practice. OBJECTIVE: We aimed to evaluate the diagnostic validity and reliability of the Montreal Cognitive Assessment (MoCA) test as a screening tool for CI in patients with multiple sclerosis, as compared against the Brief Neuropsychological Battery. MATERIAL AND METHODS: We recruited 52 patients with multiple sclerosis (61.5% women; mean age [standard deviation]: 41.7 [11.5] years). We analysed the reliability (internal consistency, interobserver reliability, and test-retest reliability), construct validity (factor analysis, Pearson correlation coefficient, and coefficient of determination), and criterion validity (ROC curve, sensitivity, specificity, total agreement, positive and negative predictive values, positive and negative likelihood ratios, and Fagan nomogram) of the MoCA test in this population. RESULTS: The prevalence of CI was 21.2% according to findings from the Brief Neuropsychological Battery, and 25% according to the MoCA test. The MoCA test showed good internal consistency (Cronbach alpha, 0.822) and interobserver and test-retest reliability (intraclass correlation coefficient 0.80 and 0.96, respectively). The correlation coefficient between total Brief Neuropsychological Battery and MoCA test scores was 0.82. The optimal cut-off point on the ROC curve was 25-26, yielding 91% sensitivity and 93% specificity. CONCLUSION: The MoCA test is a valid and reliable tool for screening for CI in patients with multiple sclerosis.

Autosomal recessive spinocerebellar ataxia SCAR8/ARCA1: First families detected in Spain. / Heredoataxia cerebelosa recesiva ARCA1/SCAR8: primeras familias detectadas en España.

INTRODUCTION: Autosomal recessive spinocerebellar ataxia type 8 (ARCA1/SCAR8) is caused by mutations of the SYNE1 gene. The disease was initially described in families from Quebec (Canada) with a phenotype of pure cerebellar syndrome, but in recent years has been reported with a more variable clinical phenotype in other countries. Cases have recently been described of muscular dystrophy, arthrogryposis, and cardiomyopathy due to SYNE1 mutations. OBJECTIVE: To describe clinical and molecular findings from 4 patients (3 men and one woman) diagnosed with ARCA1/SCAR8 from 3 Spanish families from different regions. MATERIAL AND METHODS: We describe the clinical, paraclinical, and genetic results from 4 patients diagnosed with ARCA1/SCAR8 at different Spanish neurology departments. RESULTS: Onset occurred in the third or fourth decade of live in all patients. After 15 years of progression, 3 patients presented pure cerebellar syndrome, similar to the Canadian patients; the fourth patient, with over 30 years' progression, presented vertical gaze palsy, pyramidal signs, and moderate cognitive impairment. In all patients, MRI studies showed cerebellar atrophy. The genetic study revealed distinct pathogenic SYNE1 mutations in each family. CONCLUSIONS: ARCA1/SCAR8 can be found worldwide and may be caused by many distinct mutations in the SYNE1 gene. The disease may manifest with a complex phenotype of varying severity.

Clinical management departments for the neurosciences.

INTRODUCTION: Neuroscience-related clinical management departments (UGC in Spanish) represent a means of organising hospitals to deliver patient-centred care as well as specific clinical and administrative management models. DEVELOPMENT: The authors review the different UGC models in Spain and their implementation processes as well as any functional problems. We pay special attention to departments treating neurological patients. CONCLUSIONS: Neuroscience-related specialties may offer a good framework for the units that they contain. This may be due to the inherent variability and costs associated with neurological patients, the vital level of coordination that must be present between units providing care, and probably to the dynamic nature of the neurosciences as well. Difficulties associated with implementing and gaining acceptance for the new model have limited such UGCs until now.

The social impact of Parkinson's disease in Spain: Report by the Spanish Foundation for the Brain. / Informe de la Fundación del Cerebro sobre el impacto social de la enfermedad de Parkinson en España.

INTRODUCTION: Understanding the social and economic impact of Parkinson's disease is essential for resource planning and raising social awareness. DEVELOPMENT: Researchers reviewed the data published to date on epidemiology, morbidity and mortality, dependency, and economic impact of Parkinson's disease in Spain. In addition, a study has been carried out in order to define the public and private health care resources of Spanish patients affected by Parkinson's disease by means of an e-mail survey of all neurologists specialising in this disease and belonging to the Spanish Society of Neurology's study group for movement disorders. CONCLUSIONS: The incidence and prevalence rates of Parkinson's disease in Spain are similar to those in the rest of Europe. According to current population estimates, there are at least 300.000 patients with Parkinson's disease and one new case per 10.000 habitants per year in Spain. This has a major impact on the patient's quality of life and nearly doubles patient mortality. In addition, the disease generates sizeable costs for the country that may exceed 17.000€ per year per patient; costs will rise due to the ageing of the population and the new therapies employed. Healthcare professionals and administrators dedicate their efforts to providing quality care to patients. Despite the above, we still have a long way to go in order to provide quality, efficient, multidisciplinary, and universal healthcare.

Study of outpatient neurological care in the Region of Madrid: The impact of implementing free choice of hospital.

INTRODUCTION: A new model permitting free choice of hospital has been introduced in the Region of Madrid. This may result in changes in how outpatient neurological care is provided and managed. The purpose of this study is to analyse initial visits to a general neurology department in the Region of Madrid and record the health district corresponding to each patient's residence. METHODS: Observational and prospective study of a cohort of patients making initial outpatient visits to a neurology department between 16 September 2013 and 16 January 2014. RESULTS: The study included 1109 patients (63.8% women, mean age 55.2±20.5). The most frequent diagnostic groups were periodic headache, cognitive disorders, and neuromuscular diseases. Non-neurological diseases were diagnosed in 1.1% of the cases. The mean time of delay was 7.2±5.1 days. Residents within the hospital's health district made up 73.8% of the total, while 26.2% chose a hospital outside of the health district corresponding to their residences. In the latter group, 59.5% made the choice based on the level of care offered, while 39.7% changed hospitals due to shorter times to consultation. The patients who came from another health district were younger (50.7 vs 57.3, P<.0001) and had a lower rate of discharges on the first visit (16.4% vs 30.1%, P<.0001). CONCLUSION: The model of free choice of hospital delivers significant changes in healthcare management and organisation. Reasons given for choosing another hospital are more ample experience and shorter delays with respect to the home district hospital. Management of patients from outside the health district is associated with greater complexity.

What happens to medical articles submitted in Spanish that are not accepted for publication?

INTRODUCTION: The fate of manuscripts submitted and subsequently rejected by Spanish-language journals is unknown. The present study was designed to determine whether or not articles submitted to Neurología are published following rejection, and if so, where. METHODS: We searched Medline in late April 2012 and also analysed all manuscripts rejected by Neurología between October 2004 and April 2012 according to that journal's two databases. In that period, 1277 articles were submitted to the journal. RESULTS: Of the 271 manuscripts rejected by Neurología, 54 articles (19.9%) were published in other journals. Neurology journals published 31 of the manuscripts (57.4%); 43 manuscripts (79.6%) appeared in Spanish-language journals. Of the rejected manuscripts, 24.1% of the originals, 8.3% of the letters to the editor, 28.9% of the case reports, 22.6% of the reviews and 6.3% of the images were published. Authors with three previously published articles on the same topic managed to publish their manuscripts in 34% of the cases, compared to only 11.8% of authors with fewer published articles (P < .0001). Of the total manuscripts rejected between 2004 and 2010, 24.8% were eventually published. The median time lapse between article submission and publication was 13 months (range, 2-59 months). CONCLUSION: Manuscripts rejected by Neurología are often published in other journals, but this scenario is not as common as in English-language journals. In the case of Neurología, the editor's decision to reject an article is more significant than it would be in an English-language journal because the author will have fewer additional possibilities of having the manuscript published.

Primary progressive aphasia: from syndrome to disease.

INTRODUCTION: Primary progressive aphasia (PPA) is a clinical syndrome characterised by a progressive decline in language and speech of neurodegenerative origin. Major breakthroughs made in recent years have lent us a better understanding of this syndrome, which may be the first manifestation of any of a number of neurodegenerative diseases. DEVELOPMENT: We reviewed the main aspects of PPA epidemiology, clinical manifestations, diagnosis, aetiology and treatment. Most cases manifest sporadically and the typical age of onset is between 50 and 70 years. Three clinically distinct variants have been described: nonfluent or agrammatic PPA, semantic PPA and logopenic PPA. Each of these variants tends to be associated with specific histopathological findings, but clinical diagnostic methods are imperfect predictors of underlying pathology. Anatomical and functional neuroimaging can provide useful biomarkers. Several treatments have been proposed, and while no clear benefits have been demonstrated, acetylcholinesterase inhibitors may be useful, especially in the logopenic variant. CONCLUSIONS: PPA is an emerging syndrome which may be more prevalent than we might expect. It was previously listed as part of the frontotemporal dementia spectrum, and it is also related to Alzheimer disease. Clinical diagnosis, complemented by a biomarker evaluation, may predict the underlying pathology, which in turn will improve treatment possibilities.

[Conflict of interests and scientific publications]. / Conflicto de intereses y publicaciones científicas.

INTRODUCTION: The potential relationships of interest between authors, reviewers, editors and financial management of the journals can lead to a conflict of interest in their performances. DEVELOPMENT: It analyzes the potential conflicts of interest in the papers, with extreme examples, assessing the need for careful statement of the relations, especially economic. CONCLUSIONS: Potential conflicts of interest should be transparent and the knowledge and values should be an objective of the magazines. The declaration of relationships should be required in the communication of research, but their existence should not prejudge misconduct.

[Feen report on epilepsy in Spain]. / FEEN: Informe sociosantario FEEN sobre la epilepsia en España.

INTRODUCTION: Epilepsy is a very common disease in Spain. There is a great lack of information on real epidemiological data and the patient impact of this disease. The objective of the Spanish Foundation for Neurological Diseases (FEEN) report is to collect epidemiological data, morbidity, mortality and costs of this disease in Spain. DEVELOPMENT: A search was carried out in Medline on publications up to 2010, as well as a review of data published by the Spanish National Statistics Institute (INE). There are about 400,000 patients with epilepsy in Spain. Approximately 5 -10% of the population will experience a seizure in their lifetime, and up to 20% of these will have recurrent seizures. Using hospital discharge report data, hospital admissions for epilepsy are around 35 patients per 100,000 patients. Mortality risk in epileptic patients is two or three times higher than in non-epileptics. The mean total annual cost of drug resistant epilepsy patient in Spain is 6,935 Euros. The total cost of epilepsy according to data from the year 2000 could be around 5% of the total health budget. CONCLUSIONS: It is very important to maintain disease registers. This initiative should be encouraged by the patient associations and scientific societies. This report confirms that epilepsy has a great social and health impact on the population.