Striatal and thalamic automatic segmentation, morphology, and clinical correlates in Parkinsonism: Parkinson's disease, multiple system atrophy and progressive supranuclear palsy.

Parkinson's disease (PD), multisystem atrophy (MSA), and progressive supranuclear palsy (PSP) present similarly with bradykinesia, tremor, rigidity, and cognitive impairments. Neuroimaging studies have found differential changes in the nigrostriatal pathway in these disorders, however whether the volume and shape of specific regions within this pathway can distinguish between atypical Parkinsonian disorders remains to be determined. This paper investigates striatal and thalamic volume and morphology as distinguishing biomarkers, and their relationship to neuropsychiatric symptoms. Automatic segmentation to calculate volume and shape analysis of the caudate nucleus, putamen, and thalamus were performed in 18 PD patients, 12 MSA, 15 PSP, and 20 healthy controls, then correlated with clinical measures. PSP bilateral thalami and right putamen were significantly smaller than controls, but not MSA or PD. The left caudate and putamen significantly correlated with the Neuropsychiatric Inventory total score. Bilateral thalamus, caudate, and left putamen had significantly different morphology between groups, driven by differences between PSP and healthy controls. This study demonstrated that PSP patient striatal and thalamic volume and shape are significantly different when compared with controls. Parkinsonian disorders could not be differentiated on volumetry or morphology, however there are trends for volumetric and morphological changes associated with PD, MSA, and PSP.

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

INTRODUCCIÓN: El manejo de la enfermedad de Parkinson en la mujer en edad fértil nos plantea como principal reto el manejo de la enfermedad y los fármacos durante el embarazo y lactancia. El aumento de la edad gestacional de la mujer hace más probable que la incidencia de embarazos pueda incrementarse. OBJETIVO: Definir las características clínicas y los factores que condicionan la vida de la mujer en edad fértil con enfermedad de Parkinson y definir una guía de actuación y manejo del embarazo en estas pacientes. RESULTADOS: Este documento de consenso se ha realizado mediante una búsqueda bibliográfica exhaustiva y discusión de los contenidos realizados por un grupo de expertos en trastornos del movimiento de la Sociedad Española de Neurología. CONCLUSIONES: La enfermedad de Parkinson afecta a todos los aspectos relacionados con la salud sexual y reproductiva de la mujer en edad fértil. Se debe planificar el embarazo en las mujeres con enfermedad de Parkinson para minimizar los riesgos teratogénicos sobre el feto. Se recomienda un abordaje multidisciplinar de estas pacientes para tener en cuenta todos los aspectos implicados

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

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

INTRODUCCIÓN: Muchas enfermedades que cursan con trastornos del movimiento hipercinético comienzan o afectan a mujeres en edad fértil. Es importante conocer los riesgos que tienen las mujeres con estas enfermedades durante el embarazo, así como los posibles efectos de los tratamientos sobre el feto. OBJETIVOS: Definir las características clínicas y los factores que condicionan la vida de la mujer en edad fértil con distonía, corea, síndrome de Tourette, temblor y síndrome de piernas inquietas. Definir una guía de actuación y manejo del embarazo y lactancia en las pacientes con esta enfermedad. DESARROLLO: Este documento de consenso se ha realizado mediante una búsqueda bibliográfica exhaustiva y discusión de los contenidos llevadas a cabo por un Grupo de Expertos en Trastornos del Movimiento de la Sociedad Española de Neurología (SEN). CONCLUSIONES: En todas las mujeres que padecen o comienzan con trastornos del movimiento hipercinéticos se debe valorar el riesgo-beneficio de los tratamientos, reducir al máximo la dosis eficaz o administrarlo de forma puntual en los casos en que sea posible. En aquellas enfermedades de causa hereditaria es importante un consejo genético para las familias. Es importante reconocer los trastornos del movimiento desencadenados durante el embarazo como determinadas coreas y síndrome de piernas inquietas

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

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.

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.

Encefalopatía con lesión reversible del cuerpo calloso: una entidad poco conocida con pronóstico favorable / Mild encephalitis/encephalopathy with reversible splenial lesion: A little-known entity with favourable prognosis

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La gestión de la asistencia neurológica en tiempos de la pandemia de Covid-19 / Management of neurological care during the COVID-19 pandemic

INTRODUCCIÓN: La pandemia de Covid-19 ha supuesto la necesidad de tomar decisiones para mantener la asistencia neurológica, sin precedentes. En este artículo se analiza esa toma de decisiones operativas. Desarrollo: Los autores refieren las fórmulas empleadas como son la realización de un plan de reorganización funcional, estrategias para la hospitalización y urgencias, la realización de consultas telefónicas para el mantenimiento de la atención neurológica, la asistencia en un área externa al hospital para pacientes preferentes, las decisiones sobre exploraciones complementarias y tratamientos intrahospitalarios periódicos o implantar un teléfono para la priorización de crisis epilépticas. CONCLUSIÓN: A pesar de la situación de aislamiento, los servicios de neurología deben mantener la atención de sus pacientes a través de distintas fórmulas operativas, que como cualquier elemento de gestión, deberán evaluarse

INTRODUCTION: The COVID-19 epidemic has led to the need for unprecedented decisions to be made to maintain the provision of neurological care. This article addresses operational decision-making during the epidemic. Development: We report the measures taken, including the preparation of a functional reorganisation plan, strategies for hospitalisation and emergency management, the use of telephone consultations to maintain neurological care, provision of care at a unit outside the hospital for priority patients, decisions about complementary testing and periodic in-hospital treatments, and the use of a specific telephone service to prioritise patients with epileptic seizures. CONCLUSION: Despite the situation of confinement, neurology departments must continue to provide patient care through different means of operation. Like all elements of management, these must be evaluated

Management of neurological care during the COVID-19 pandemic. / La gestión de la asistencia neurológica en tiempos de la pandemia de Covid-19.

INTRODUCTION: The COVID-19 epidemic has led to the need for unprecedented decisions to be made to maintain the provision of neurological care. This article addresses operational decision-making during the epidemic. DEVELOPMENT: We report the measures taken, including the preparation of a functional reorganisation plan, strategies for hospitalisation and emergency management, the use of telephone consultations to maintain neurological care, provision of care at a unit outside the hospital for priority patients, decisions about complementary testing and periodic in-hospital treatments, and the use of a specific telephone service to prioritise patients with epileptic seizures. CONCLUSION: Despite the situation of confinement, neurology departments must continue to provide patient care through different means of operation. Like all elements of management, these must be evaluated.

Polaquiuria como tic motor complejo en un paciente con síndrome de Tourette / Pollakiuria as a complex motor tic in a patient with Tourette síndrome

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The high prevalence of impulse control behaviors in patients with early-onset Parkinson's disease: A cross-sectional multicenter study.

INTRODUCTION: In Parkinson's disease patients, impulse control disorders (ICDs) have been associated with younger age and early disease onset, yet the prevalence of ICDs in early-onset Parkinson's disease (EOPD) patients has yet to be studied. Thus, we set out to compare the prevalence of impulse control behaviors (ICBs) in a cohort of EOPD patients with that in age and gender matched healthy controls (HCs), as well as to analyze the association of these symptoms with the use of dopaminergic drugs and other clinical or demographic factors. METHODS: A cross-sectional, multicenter study was carried out on patients recruited from outpatient Movement Disorder Clinics, assessing ICBs using the short form of the Questionnaire for Impulsive-Compulsive Disorders in Parkinson's Disease (QUIP). In addition, depression and quality of life (QoL) were measured, along with other demographic and clinical variables. RESULTS: Of the 87 EOPD patients, 49 (58.3%) displayed an ICB, as did 28 of the 87 HCs (32.9%; p=0.001). Most of the EOPD patients that displayed an ICB (91.8%) were medicated with a dopamine agonist (DA) and accordingly, DA treatment was associated with a 7-fold increased risk of developing an ICB. Patients with ICBs had a higher depression score and a worse QoL. CONCLUSIONS: ICBs are much more prevalent in EOPD patients than in HCs and they are associated with DA intake, depression and a worse QoL.

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

INTRODUCCIÓN: Conocer el alcance socieoeconómico de la enfermedad de Parkinson es esencial para la planificación de recursos y concienciación social. Desarrollo: Se ha realizado una revisión de los datos publicados hasta el momento sobre epidemiología, morbimortalidad, dependencia e impacto económico de la enfermedad de Parkinson en España. Además se ha llevado a cabo un estudio cuyo objetivo principal ha sido definir los recursos asistenciales públicos y privados que tienen los afectados por la enfermedad de Parkinson en nuestro país mediante una encuesta por mail a todos los neurólogos con dedicación especial a esta enfermedad, pertenecientes al grupo de Trastornos del Movimiento de la Sociedad Española de Neurología. CONCLUSIONES: La enfermedad de Parkinson en España tiene una incidencia y prevalencia similar al resto de Europa. Con la estimación de población actual se obtiene que debe haber en España al menos 300.000 pacientes con enfermedad de Parkinson y a al menos un nuevo caso por 10.000 habitantes año. Esta produce gran impacto en la calidad de vida del paciente y aumenta a casi el doble la mortalidad de los pacientes. Además supone un coste económico muy importante para el país, que puede llegar hasta más de 17.000 € anuales por paciente y que con el envejecimiento de la población y las nuevas terapias va a ir incrementándose. Los profesionales y administraciones realizan un gran esfuerzo para proporcionar una asistencia de calidad a los pacientes. A pesar de ello es mucho el camino que nos queda por recorrer para que una asistencia de calidad, eficaz y multidisciplinar sea universal para todos los pacientes con esta enfermedad

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

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.

[Galantamine therapy in dementia associated with CADASIL]. / Tratamiento con galantamina en la demencia asociada a CADASIL.

INTRODUCTION: Cholinergic neuronal impairment has been suggested in cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencefalopathy (CADASIL). Cholinomimetic therapy could be useful. CASE REPORTS: Four patients with CADASIL and dementia were treated with the acetylcholinesterase inhibitor galantamine and we assessed cognitive, behavioral, functional and the caregiver burden aspects. Three patients showed either mild improvement or stabilization in the behavior and caregiver burden. CONCLUSION: Our results suggest some benefit from galantamine treatment and they could support the existence of a cholinergic deficit in CADASIL.

Tratamiento con galantamina en la demencia asociada a CADASIL / Galantamine therapy in dementia associated with cadasil

Introducción. Se ha sugerido que en la arteriopatía cerebral autosómica dominante con infartos subcorticales y leucoencefalopatía (CADASIL) existe un déficit colinérgico neuronal. La terapia colinomimética podría ser útil. Casos clínicos. Cuatro pacientes con CADASIL y demencia fueron tratados con el inhibidor de acetilcolinesterasa galantamina y se valoraron los aspectos cognitivos, conductuales, funcionales y de sobrecarga del cuidador. Tres pacientes mostraron una leve mejoría o una estabilización de las alteraciones conductuales y de la sobrecarga del cuidador. Conclusión. Nuestros resultados sugieren algún beneficio del tratamiento con galantamina y apoyarían la teoría del déficit colinérgico en la CADASIL

Introduction. Cholinergic neuronal impairment has been suggested in cerebral autosomal dominant arteriopathywith subcortical infarcts and leucoencefalopathy (CADASIL). Cholinomimetic therapy could be useful. Case reports. Four patients with CADASIL and dementia were treated with the acetylcholinesterase inhibitor galantamine and we assessed cognitive, behavioral, functional and the caregiver burden aspects. Three patients showed either mild improvement orstabilization in the behavior and caregiver burden. Conclusion. Our results suggest some benefit from galantamine treatment and they could support the existence of a cholinergic deficit in CADASIL

Linfomatosis cerebral como causa de leucoencefalopatía / Lymphomatosis cerebri as the cause of leukoencephalopathy

La linfomatosis cerebral (LC) es un tipo poco frecuente de linfoma primario del sistema nervioso centralcaracterizado por la afectación difusa e infiltrante de la sustancia blanca cerebral sin formar una masa. Objetivo. Presentar el caso de una paciente con LC para llamar la atención sobre la existencia de esta enfermedad, raramente diagnosticada, y sobre su presentación inicial en la resonancia magnética (RM) como leucoencefalopatía. Caso clínico. Mujer de 56 años que inició un cuadro de demencia subaguda. La tomografía axial computarizada y la RM craneal revelaron una afectación extensa, difusa y bilateral de la sustancia blanca, núcleos basales, mesencéfalo y protuberancia, sin efecto masa ni captación de contraste. Biopsia estereotáxica de la sustancia blanca, no concluyente, que mostró un infiltrado inflamatorio mononuclearmixto, de células B y T, de disposición perivascular. No se observó atipia citológica. Se realizó tratamiento con corticoides, con lo que se obtuvo mejoría clínica y radiológica en los dos primeros meses. Al siguiente mes se produjo un deterioro clínico rápido con somnolencia y empeoramiento de la marcha. La RM mostró un aspecto más heterogéneo de la lesión, con efecto masa sobre estructuras adyacentes y realce parcheado del contraste. Una biopsia cerebral en cuña llevó al diagnóstico de linfoma de células B de alto grado. Conclusiones. La apariencia radiológica e histológica de la LC puede no ser la habitual de los linfomas primarios del sistema nervioso central, y su presentación clínica puede ser similar a la de otros procesos difusos de afectación de la sustancia blanca (gliomatosis cerebral, enfermedades inflamatorias de la sustancia blanca como la enfermedad de Behçet, la enfermedad de Sjögren o el lupus eritematoso sistémico)

Lymphomatosis cerebri (LC) is an infrequent type of primary lymphoma of the central nervous systemthat is characterised by diffuse, infiltrating involvement of the white matter of the brain without the formation of a mass. Aim. To report the case of a patient with LC in order to draw attention to this disease, which is rarely diagnosed, and to its initialpresentation in magnetic resonance imaging (MRI) as leukoencephalopathy. Case report. Our patient was a 56-year-old female who had clinical signs and symptoms of sub-acute dementia. Computerised axial tomography and MRI of the head revealed extensive, diffuse and bilateral involvement of the white matter, basal nuclei, mesencephalon and pons, with no masseffect or contrast enhancement. A stereotactic biopsy of the white matter (which was not conclusive) showed a perivascular mixed mononuclear-cell inflammatory infiltrate of B and T cells. No cytologic atypia was observed. Treatment was establishedwith corticoids, which produced a clinical and radiological improvement in the first two months. During the next month the patient underwent rapid clinical deterioration with sleepiness and a worsening of the ability to walk. In an MRI scan the lesion had a more heterogeneous appearance with mass effect on adjacent structures and patchy contrast enhancement. A wedge biopsy of brain tissue led to a diagnosis of high-grade B-cell lymphoma. Conclusions. The imaging and histological appearance of LC may not be the one typically found in primary lymphomas of the central nervous system, and its clinical presentation may be similar to that of other diffuse processes involving compromise of the white matter (cerebral gliomatosis, inflammatory diseases of the white matter, such as Behçet’s disease, Sjögren’s disease or systemic lupus erythematosus)

[Lymphomatosis cerebri as the cause of leukoencephalopathy]. / Linfomatosis cerebral como causa de leucoencefalopatía.

INTRODUCTION: Lymphomatosis cerebri (LC) is an infrequent type of primary lymphoma of the central nervous system that is characterised by diffuse, infiltrating involvement of the white matter of the brain without the formation of a mass. AIM: To report the case of a patient with LC in order to draw attention to this disease, which is rarely diagnosed, and to its initial presentation in magnetic resonance imaging (MRI) as leukoencephalopathy. CASE REPORT: Our patient was a 56-year-old female who had clinical signs and symptoms of sub-acute dementia. Computerised axial tomography and MRI of the head revealed extensive, diffuse and bilateral involvement of the white matter, basal nuclei, mesencephalon and pons, with no mass effect or contrast enhancement. A stereotactic biopsy of the white matter (which was not conclusive) showed a perivascular mixed mononuclear-cell inflammatory infiltrate of B and T cells. No cytologic atypia was observed. Treatment was established with corticoids, which produced a clinical and radiological improvement in the first two months. During the next month the patient underwent rapid clinical deterioration with sleepiness and a worsening of the ability to walk. In an MRI scan the lesion had a more heterogeneous appearance with mass effect on adjacent structures and patchy contrast enhancement. A wedge biopsy of brain tissue led to a diagnosis of high-grade B-cell lymphoma. CONCLUSIONS: The imaging and histological appearance of LC may not be the one typically found in primary lymphomas of the central nervous system, and its clinical presentation may be similar to that of other diffuse processes involving compromise of the white matter (cerebral gliomatosis, inflammatory diseases of the white matter, such as Behçet's disease, Sjögren's disease or systemic lupus erythematosus).

Enfermedad de Whipple cerebral. Un caso clínico con confirmación histológica necrópsica / Whipple's disease coonfined to the brain. A clinical case with pathological confirmation at necropsy

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