Epilepsy in elderly patients: does age of onset make a difference?

INTRODUCTION: Epilepsy is most frequent in children and elderly people. Today's population is ageing and epilepsy prevalence is increasing. The type of epilepsy and its management change with age. METHODS: We performed a retrospective, observational study comparing patients aged ≥ 65 years with epilepsy diagnosed before and after the age of 65, and describing epilepsy characteristics and comorbidities in each group. RESULTS: The sample included 123 patients, of whom 61 were diagnosed at < 65 years of age (group A), 62 at ≥ 65 of age (group B). Sex distribution was similar in both groups, with 39 men (62.9%) in group A and 37 (60.7%) in group B. Mean age was 69.97 ±â€¯5.6 years in group A and 77.29 ±â€¯6.73 in group B. The most common aetiology was cryptogenic in group A (44.3%, n = 27) and vascular in group B (74.2%, n = 46). History of stroke was present in 12 patients from group A (19.7%) and 32 (51.6%) in group B. Antiepileptic drugs were prescribed at lower doses in group A. Statistically significant differences were found between groups for history of ischaemic stroke, cognitive impairment, psychiatric disorders, and diabetes mellitus; degree of dependence; and number of antiepileptic drugs. CONCLUSION: Age of onset ≥ 65 years is closely related to cardiovascular risk factors; these patients require fewer antiepileptic drugs and respond to lower doses. Some cases initially present as status epilepticus.

Headache as a reason for consultation: the primary care perspective.

INTRODUCTION: Headache is a frequent reason for consultation with primary care (PC) physicians. However, the condition is underdiagnosed and undertreated. One reason for this may be the lack of specific training on headache in PC. METHODS: We conducted a descriptive cross-sectional study of data gathered with a self-administered survey that was completed by PC physicians from our health district. We collected sociodemographic data and information on previous training in neurology and headache, and training needs. RESULTS: The survey was completed by 104 PC physicians, 53% of whom were women; mean age was 49 years. Most respondents worked in urban settings (42.3%) and had been trained via residency (77.9%). Although 65.4% spent more time with patients with headache than with other patients, only 32.7% used the International Classification of Headache Disorders. In our sample, 68.3% of respondents reported a high or very high interest in headache, and 75.9% wished to receive further training on the condition; theoretical and practical courses and update lectures were regarded as the most useful tools. In clinical practice, 90% used triptans and 78% used preventive treatments. The most frequently used drug was amitriptyline; only 22.1% choose topiramate. CONCLUSIONS: PC physicians are in frequent contact with patients with headache and show interest in receiving training on this condition. This could be helpful in designing training programmes aimed at improving quality of care in this area.

Cefalea como motivo de consulta: la visión desde atención primaria / Headache as a reason for consultation: the primary care perspective

Introducción: La cefalea es un motivo de consulta frecuente en atención primaria (AP), sin embargo, se trata de una patología infradiagnosticada e infratratada. Uno de los posibles factores es la falta de formación de los profesionales de AP en cefaleas.MétodosEstudio descriptivo transversal mediante encuesta autoadministrada a los médicos de AP de nuestro sector sanitario. Se recogieron variables sociodemográficas, variables relacionadas con la formación previa en neurología y cefaleas y variables en relación con sus necesidades formativas.ResultadosCompletaron la encuesta 104 médicos de AP, de los cuales el 53% fueron mujeres y la edad media fue 49 años. La mayoría de los encuestados trabajan en un centro de salud urbano (42,3%) y se han formado vía MIR (77,9%). El 65,4% dedica más tiempo a una consulta de cefaleas que a otras consultas y solo un 32,7% utiliza la Clasificación Internacional de Cefaleas. El 68,3% declara tener un interés alto o muy alto en cefaleas y al 75,9% le gustaría mejorar su formación, eligiendo cursos teórico-prácticos y ponencias de actualización como la herramienta más útil. Un 90% utiliza triptanes en su práctica clínica diaria y el 78% emplea tratamientos preventivos. El fármaco más utilizado es amitriptilina y solo un 22,1% elige topiramato.ConclusionesLos profesionales de AP están en contacto constante con pacientes con cefaleas y muestran interés en la formación en esta patología, lo que podría ayudarnos a diseñar programas docentes dirigidos a mejorar la calidad asistencial en este ámbito. (AU)

Introduction: Headache is a frequent reason for consultation with primary care (PC) physicians. However, the condition is underdiagnosed and undertreated. One reason for this may be the lack of specific training on headache in PC.MethodsWe conducted a descriptive cross-sectional study of data gathered with a self-administered survey that was completed by PC physicians from our health district. We collected sociodemographic data and information on previous training in neurology and headache, and training needs.ResultsThe survey was completed by 104 PC physicians, 53% of whom were women; mean age was 49 years. Most respondents worked in urban settings (42.3%) and had been trained via residency (77.9%). Although 65.4% spent more time with patients with headache than with other patients, only 32.7% used the International Classification of Headache Disorders. In our sample, 68.3% of respondents reported a high or very high interest in headache, and 75.9% wished to receive further training on the condition; theoretical and practical courses and update lectures were regarded as the most useful tools. In clinical practice, 90% used triptans and 78% used preventive treatments. The most frequently used drug was amitriptyline; only 22.1% choose topiramate.ConclusionsPC physicians are in frequent contact with patients with headache and show interest in receiving training on this condition. This could be helpful in designing training programmes aimed at improving quality of care in this area. (AU)

[Epidemiology of migraine in Spain and Latin America]. / Epidemiología de la migraña en España y Latinoamérica.

INTRODUCTION: Migraine is a very disabling disease that has a great impact on patients' quality of life and interferes in their personal, social, work and family spheres. From a historical point of view, the connection between the Iberian Peninsula and Latin America has been very important, and so it seems reasonable to find there are parallels in the epidemiology of this disease, given the role that certain genetic and lifestyle-related determinants have in its natural history. AIM: To conduct a detailed review of the descriptive epidemiological studies of migraine in Spain and Latin America. DEVELOPMENT: Literature search of epidemiological studies on migraine in our country and in Latin America. The population studied, the methodology, the questionnaire used for diagnosis and the prevalence data were analysed. Altogether 23 studies were evaluated. CONCLUSIONS: Not all countries have population-based epidemiological studies of migraine, and most of them were conducted more than 10 years ago. Moreover, a wide range of methodologies were applied. The prevalence data obtained in the selected studies, with the exception of some conducted in Brazil and Peru, are very similar to those found in Spain.

TITLE: Epidemiología de la migraña en España y Latinoamérica.Introducción. La migraña es una enfermedad muy invalidante con un gran impacto en la calidad de vida del paciente e interferencia en su esfera personal, social, laboral y familiar. Desde un punto de vista histórico, la conexión entre la Península Ibérica y Latinoamérica ha sido muy importante, por lo que parece razonable que existan paralelismos en la epidemiología de esta enfermedad, dado el papel que determinados condicionantes genéticos y relacionados con el estilo de vida tienen en su historia natural. Objetivo. Revisar de forma detallada los estudios epidemiológicos descriptivos de la migraña en España y América Latina. Desarrollo. Búsqueda bibliográfica de estudios de epidemiología sobre migraña en nuestro país y en los que conforman Latinoamérica. Se analiza la población estudiada, la metodología, el cuestionario utilizado para el diagnóstico y los datos de prevalencia. Se evaluaron un total de 23 estudios. Conclusiones. No todos los países cuentan con estudios epidemiológicos de migraña de base poblacional y la mayor parte de ellos se desarrollaron hace más de diez años. La metodología aplicada es, además, muy heterogénea. Los datos de prevalencia obtenidos en los estudios seleccionados, con la excepción de algunos realizados en Brasil y Perú, son muy similares a los encontrados en España.

Utilidad del electroencefalograma en el manejo de la epilepsia en el Servicio de Urgencias / Usefulness of electroencephalography for the management of epilepsy in emergency departments

INTRODUCCIÓN: El electroencefalograma (EEG) es una prueba diagnóstica esencial en epilepsia. Su uso en los Servicios de Urgencias (SU) es limitado, estando habitualmente restringido al diagnóstico y el manejo del estado epiléptico no convulsivo (EENC). Sin embargo, pueden existir otras situaciones en las que, por su perfil temporal, el EEG puede ser una herramienta útil en este entorno. MÉTODOS: Estudio observacional retrospectivo, sobre la base de la historia clínica, de los pacientes atendidos en el SU de nuestro hospital por crisis epilépticas (CE) y sospecha de EENC a los que se realizó un EEG, en el periodo 2015-2016, recogiendo variables demográficas y clínicas. RESULTADOS: Se reclutó a 87 pacientes, con una edad media de 44 años. El motivo de consulta más frecuente fue CE, el 59,8% por primera CE (pCE) y el 27,6% por CE en paciente con epilepsia; en 12,6% la sospecha era EENC. Se observaron descargas epileptiformes interictales (DEI) en el 38,4% de los pacientes atendidos por pCE y en el 33,3% de los epilépticos conocidos; el 36,4% de los posibles EENC se confirmaron mediante EEG. El EEG con DEI supuso un inicio o cambio de tratamiento crónico en el 59,8% de los pacientes. CONCLUSIONES: El EEG es una herramienta útil en el manejo de las CE en los SU, tanto de situaciones agudas graves como el EENC, como en el estudio diagnóstico de la epilepsia de debut o no filiada

INTRODUCTION: Electroencephalography (EEG) is an essential diagnostic tool in epilepsy. Its use in emergency departments (ED) is usually restricted to the diagnosis and management of non-convulsive status epilepticus (NCSE). However, EDs may also benefit from EEG in the context of other situations in epilepsy. METHODS: We conducted a retrospective observational study using the clinical histories of patients treated at our hospital's ED for epileptic seizures and suspicion of NCSE and undergoing EEG studies in 2015 and 2016. We collected a series of demographic and clinical variables. RESULTS: Our sample included 87 patients (mean age of 44 years). Epileptic seizures constituted the most common reason for consultation: 59.8% due to the first episode of epileptic seizures (FES), 27.6% due to recurrence, and 12.6% due to suspected NCSE. Interictal epileptiform discharges (IED) were observed in 38.4% of patients reporting FES and in 33.3% of those with a known diagnosis of epilepsy. NCSE was confirmed by EEG in 36.4% of all cases of suspected NCSE. Presence of IED led to administration of or changes in long-term treatment in 59.8% of the patients. CONCLUSIONS: EEG is a useful tool for seizure management in EDs, not only for severe, sudden-onset clinical situations such as NCSE but also for diagnosis in cases of non-affiliated epilepsy and in patients experiencing the first episode of epilepsy

Epilepsia mioclónica juvenil: pronóstico a largo plazo y retirada de tratamiento / Juvenile myoclonic epilepsy: long-term prognosis and antiepileptic drug withdrawal

FUNDAMENTO: La epilepsia mioclónica juvenil (EMJ) es un síndrome epiléptico clásico que carece de consenso pleno sobre la posibilidad de suspensión de tratamiento con fármacos antiepilépticos (FAE). MÉTODO: Estudio observacional, retrospectivo de una serie de pacientes con diagnóstico de EMJ de más de 20 años de evolución, atendiendo a la evolución de aquellos con retirada de FAE. RESULTADOS: Se estudiaron veinte pacientes (edad media 44,1 años, 55% hombres) con EMJ de 30 años de evolución media y edad media al inicio de 14,2 años. El tipo de crisis motora más frecuente fue la combinación de mioclónicas y tónico-clónicas (70%); el 60% de los pacientes llevan más de cinco años libres de crisis. A cuatro pacientes (20%) se les suspendieron los FAE, a dos de ellos con edad media 23 años y un tiempo medio libre de crisis de 7,5 años, que recayeron, y a los otros dos con edad media 39 años y tras 23,5 años libres de crisis, que llevan dos y nueve años sin crisis en la actualidad. CONCLUSIONES: Existe la posibilidad de suspender FAE en pacientes con EMJ con libertad de crisis mantenida en el tiempo. Sugerimos tener en cuenta la edad de suspensión y la existencia previa de un tiempo prolongado de libertad de crisis

BACKGROUND: Juvenile myoclonic epilepsy (JME) is a classic epileptic syndrome that lacks consensus on the possibility of suspending treatment with antiepileptic drugs (AD). METHOD: Retrospective observational study of a series of patients diagnosed with JME with 20 years or more of evolution, focusing on those with withdrawal from AD. RESULTS: The study involved twenty patients (average age 44.1 years, 55% men) with JME of 30 years average evolution and average age at its outset of 14.2 years. The most frequent type of motor crisis was the combination of myoclonic and tonic-clonic seizures (70%); 60% of the patients have been free of seizures for more than five years. Four patients (20%) were withdrawn from AD, two of them with an average age of 23 years and an average time free of seizures of 7.5 years, who relapsed, and the other two with an average age of 39 years and following 23.5 years free of seizures, who currently have been without seizures for two and nine years. CONCLUSIONS: The possibility of withdrawing AD in patients with JME who have been free of seizures over an extended time seems feasible. We suggest taking into account age at withdrawal and prior existence of a prolonged period of time free of seizures

[Juvenile myoclonic epilepsy: long-term prognosis and antiepileptic drug withdrawal]. / Epilepsia mioclónica juvenil: pronóstico a largo plazo y retirada de tratamiento.

BACKGROUND: Juvenile myoclonic epilepsy (JME) is a classic epileptic syndrome that lacks consensus on the possibility of suspending treatment with antiepileptic drugs (AD). METHOD: Retrospective observational study of a series of patients diagnosed with JME with 20 years or more of evolution, focusing on those with withdrawal from AD. RESULTS: The study involved twenty patients (average age 44.1 years, 55% men) with JME of 30 years average evolution and average age at its outset of 14.2 years. The most frequent type of motor crisis was the combination of myoclonic and tonic-clonic seizures (70%); 60% of the patients have been free of seizures for more than five years. Four patients (20%) were withdrawn from AD, two of them with an average age of 23 years and an average time free of seizures of 7.5 years, who relapsed, and the other two with an average age of 39 years and following 23.5 years free of seizures, who currently have been without seizures for two and nine years. CONCLUSIONS: The possibility of withdrawing AD in patients with JME who have been free of seizures over an extended time seems feasible. We suggest taking into account age at withdrawal and prior existence of a prolonged period of time free of seizures.

Nueva mutación patogénica de corea-acantocitosis / New pathogenic mutation of chorea-acanthocytosis

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Usefulness of electroencephalography for the management of epilepsy in emergency departments. / Utilidad del electroencefalograma en el manejo de la epilepsia en el Servicio de Urgencias.

INTRODUCTION: Electroencephalography (EEG) is an essential diagnostic tool in epilepsy. Its use in emergency departments (ED) is usually restricted to the diagnosis and management of non-convulsive status epilepticus (NCSE). However, EDs may also benefit from EEG in the context of other situations in epilepsy. METHODS: We conducted a retrospective observational study using the clinical histories of patients treated at our hospital's ED for epileptic seizures and suspicion of NCSE and undergoing EEG studies in 2015 and 2016. We collected a series of demographic and clinical variables. RESULTS: Our sample included 87 patients (mean age of 44 years). Epileptic seizures constituted the most common reason for consultation: 59.8% due to the first episode of epileptic seizures (FES), 27.6% due to recurrence, and 12.6% due to suspected NCSE. Interictal epileptiform discharges (IED) were observed in 38.4% of patients reporting FES and in 33.3% of those with a known diagnosis of epilepsy. NCSE was confirmed by EEG in 36.4% of all cases of suspected NCSE. Presence of IED led to administration of or changes in long-term treatment in 59.8% of the patients. CONCLUSIONS: EEG is a useful tool for seizure management in EDs, not only for severe, sudden-onset clinical situations such as NCSE but also for diagnosis in cases of non-affiliated epilepsy and in patients experiencing the first episode of epilepsy.

Cefalea post-stent carotídeo / Headache after carotid artery stenting

Introducción y objetivos: La cefalea post-stent carotídeo es aquella cefalea de aparición durante el procedimiento o en las horas posteriores, sin que haya datos de la existencia de una complicación del mismo. El objetivo de este estudio es definir las características de esta cefalea a partir de nuestra experiencia clínica. Pacientes y método: Estudio transversal prospectivo observacional de una muestra de pacientes sometidos a stenting carotídeo en el Hospital Clínico Universitario Lozano Blesa. Se estudiaron variables sociodemográficas, factores de riesgo cardiovascular, afectación carotídea y antecedentes de cefalea primaria. Se realizó una entrevista estructurada antes del procedimiento y en las siguientes 24h. Resultados: Se incluyeron un total de 56 pacientes con una media de edad de 67 ± 9,52 años; un 84% eran hombres. La cefalea apareció en 12 pacientes (21,4%), siendo el 83,3% hombres con una media de edad de 60,58 ± 9,31 años. En el 58,4% (n = 7) comenzó en las primeras 6h y en el 33,3% (n = 4) durante el procedimiento. En el 33,3% (n = 4) la duración fue menor de 10min, y en el 41,7% (n = 5) osciló entre 10-120 min. Localizaron el dolor en la región frontotemporal el 58,3% (n = 7); el 58,3% (n = 7) lo describe como unilateral. Es opresivo en un 66,7% (n = 8), de intensidad moderada en un 50% (n = 6) y el 75% de los pacientes no precisó analgesia. No se observó ninguna relación estadísticamente significativa con las variables estudiadas, excepto la edad (p = 0,007). Conclusiones: En nuestra serie, la cefalea post-stent carotídeo es de intensidad leve-moderada, unilateral, opresiva y de breve duración. Si bien son necesarios más estudios para poder definir mejor sus características y factores de riesgo

Introduction and objectives: Headache after carotid artery stenting is a headache with onset during the procedure or in the first few hours after it, and where there is no evidence to suggest a complication of that procedure. The purpose of this study is to describe the main features of these headaches based on our clinical experience. Patients and methods: Observational prospective study of a sample of patients undergoing carotid artery stenting at Hospital Clínico Universitario Lozano Blesa, in Zaragoza, Spain. We recorded sociodemographic characteristics, cardiovascular risk factors, carotid artery disease, and history of primary headache; data were gathered using structured interviews completed before and 24 hours after the procedure. Results: We included 56 patients (mean age 67 ± 9.52 years); 84% were men. Twelve patients (21.4%) experienced headache, 83.3% of whom were men; mean age was 60.58 ± 9.31 years. Headache appeared within the first 6hours in 7 patients (58.4%) and during the procedure in 4 (33.3%). Pain lasted less than 10 minutes in 4 patients (33.3%) and between 10 and 120 minutes in 5 (41.7%). Headache affected the frontotemporal area in 7 patients (58.3%); 7 patients (58.3%) described pain as unilateral. It was oppressive in 8 patients (66.7%) and of moderate intensity in 6 (50%). Nine patients (75%) required no analgesics. We found no statistically significant associations with any of the variables except for age (p = .007; t test). Conclusions: In our sample, headache after carotid artery stenting was mild to moderate in intensity, unilateral, oppressive, and short-lasting. Further studies are necessary to gain a deeper knowledge of its characteristics and associated risk factors

Epilepsy in elderly patients: does age of onset make a difference? / Epilepsia en el anciano: ¿la edad de inicio marca la diferencia?

INTRODUCTION: Epilepsy is most frequent in children and elderly people. Today's population is ageing and epilepsy prevalence is increasing. The type of epilepsy and its management change with age. METHODS: We performed a retrospective, observational study comparing patients aged ≥ 65 years with epilepsy diagnosed before and after the age of 65, and describing epilepsy characteristics and comorbidities in each group. RESULTS: The sample included 123 patients, of whom 61 were diagnosed at <65 years of age (group A), 62 at ≥ 65 of age (group B). Sex distribution was similar in both groups, with 39 men (62.9%) in group A and 37 (60.7%) in group B. Mean age was 69.97±5.6 years in group A and 77.29±6.73 in group B. The most common aetiology was unknown in group A (44.3%, n=27) and vascular in group B (74.2%, n=46). History of stroke was present in 12 patients from group A (19.7%) and 32 (51.6%) in group B. Antiepileptic drugs were prescribed at lower doses in group A. Statistically significant differences were found between groups for history of ischaemic stroke, cognitive impairment, psychiatric disorders, and diabetes mellitus; degree of dependence; and number of antiepileptic drugs. CONCLUSION: Age of onset ≥ 65 years is closely related to cardiovascular risk factors; these patients require fewer antiepileptic drugs and respond to lower doses. Some cases initially present as status epilepticus.

[Isolated hand palsy due to an ischaemic stroke]. / Paralisis aislada de la mano debido a un infarto isquemico.

TITLE: Paralisis aislada de la mano debido a un infarto isquemico.

Parálisis aislada de la mano debido a un infarto isquémico / Isolated hand palsy due to an ischaemic stroke

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Headache after carotid artery stenting. / Cefalea post-stent carotídeo.

INTRODUCTION AND OBJECTIVES: Headache after carotid artery stenting is a headache with onset during the procedure or in the first few hours after it, and where there is no evidence to suggest a complication of that procedure. The purpose of this study is to describe the main features of these headaches based on our clinical experience. PATIENTS AND METHODS: Observational prospective study of a sample of patients undergoing carotid artery stenting at Hospital Clínico Universitario Lozano Blesa, in Zaragoza, Spain. We recorded sociodemographic characteristics, cardiovascular risk factors, carotid artery disease, and history of primary headache; data were gathered using structured interviews completed before and 24hours after the procedure. RESULTS: We included 56 patients (mean age 67±9.52 years); 84% were men. Twelve patients (21.4%) experienced headache, 83.3% of whom were men; mean age was 60.58±9.31 years. Headache appeared within the first 6hours in 7 patients (58.4%) and during the procedure in 4 (33.3%). Pain lasted less than 10minutes in 4 patients (33.3%) and between 10 and 120minutes in 5 (41.7%). Headache affected the frontotemporal area in 7 patients (58.3%); 7 patients (58.3%) described pain as unilateral. It was oppressive in 8 patients (66.7%) and of moderate intensity in 6 (50%). Nine patients (75%) required no analgesics. We found no statistically significant associations with any of the variables except for age (P=.007; t test). CONCLUSIONS: In our sample, headache after carotid artery stenting was mild to moderate in intensity, unilateral, oppressive, and short-lasting. Further studies are necessary to gain a deeper knowledge of its characteristics and associated risk factors.

Headache as a reason for consultation: the primary care perspective. / Cefalea como motivo de consulta: la visión desde atención primaria.

INTRODUCTION: Headache is a frequent reason for consultation with primary care (PC) physicians. However, the condition is underdiagnosed and undertreated. One reason for this may be the lack of specific training on headache in PC. METHODS: We conducted a descriptive cross-sectional study of data gathered with a self-administered survey that was completed by PC physicians from our health district. We collected sociodemographic data and information on previous training in neurology and headache, and training needs. RESULTS: The survey was completed by 104 PC physicians, 53% of whom were women; mean age was 49 years. Most respondents worked in urban settings (42.3%) and had been trained via residency (77.9%). Although 65.4% spent more time with patients with headache than with other patients, only 32.7% used the International Classification of Headache Disorders. In our sample, 68.3% of respondents reported a high or very high interest in headache, and 75.9% wished to receive further training on the condition; theoretical and practical courses and update lectures were regarded as the most useful tools. In clinical practice, 90% used triptans and 78% used preventive treatments. The most frequently used drug was amitriptyline; only 22.1% choose topiramate. CONCLUSIONS: PC physicians are in frequent contact with patients with headache and show interest in receiving training on this condition. This could be helpful in designing training programmes aimed at improving quality of care in this area.

[Laryngeal dystonia: novel forms of therapeutic administration of botulinum toxin by direct routes]. / Distonia laringea: nuevas formas de administracion terapeutica de toxina botulinica por via directa.

AIM: To describe our experience in the treatment of laryngeal dystonia (in abduction and adduction), with special emphasis given to the technical aspects (approach procedure, dosage and type of botulinum toxin type A used), as well as treatment response and possible side effects. PATIENTS AND METHODS: We conducted a cross-sectional descriptive study of a sample of patients with laryngeal dystonia treated by means of transoral administration of onabotulinumtoxinA or incobotulinumtoxinA over a period of 10 years (2007-2017). Data collected include demographic and clinical variables, treatment response (based on a self-rating scale), the duration of treatment and the appearance of side effects. SAMPLE SIZE: 15 patients (11 women; mean age: 44.06 years) with laryngeal dystonia (mean time since onset of 40 months; 12 patients with dystonia in adduction) and 174 administrations (92% incobotulinumtoxinA; average dosage of 5 U in each vocal cord). The procedure took an average of 11.7 minutes to perform. Response was good in 31% of the procedures and very good in 57.5%. Side effects were recorded in 14.4% of the procedures, although always mild and transitory, with a predominance of dysphagia and dysphonia. CONCLUSION: In our experience, transoral administration of botulinum toxin type A to treat laryngeal dystonia has proved to be a simple, quick, effective and safe technique.

TITLE: Distonia laringea: nuevas formas de administracion terapeutica de toxina botulinica por via directa.Objetivo. Describir nuestra experiencia en el tratamiento de la distonia laringea (en abduccion y aduccion), destacando los aspectos tecnicos (procedimiento de abordaje, dosis y tipo de toxina botulinica de tipo A utilizada), asi como la respuesta al tratamiento y los posibles efectos adversos. Pacientes y metodos. Estudio descriptivo transversal de una muestra de pacientes con distonia laringea tratados mediante administracion transoral de onabotulinumtoxina o incobotulinumtoxina A durante un periodo de 10 años (2007-2017). Se recogen las variables demograficas y clinicas, la respuesta al tratamiento (a partir de una escala de autoevaluacion), la duracion de este y la aparicion de efectos adversos. Resultados. Tamaño muestral: 15 pacientes (11 mujeres; edad media: 44,06 años) con distonia laringea (tiempo medio de evolucion de 40 meses; 12 pacientes con distonia en aduccion) y 174 administraciones (92% incobotulinumtoxina A; dosis media de 5 U en cada cuerda vocal). La duracion media del procedimiento fue de 11,7 minutos. La respuesta fue notable en el 31% de los procedimientos y alta en el 57,5%. Se registraron efectos adversos en el 14,4% de los procedimientos, siempre de caracter leve y transitorio, con predominio de la disfagia y la disfonia. Conclusion. En nuestra experiencia, la administracion transoral de toxina botulinica de tipo A como tratamiento de la distonia laringea ha demostrado ser una tecnica sencilla, rapida, eficaz y segura.

Neurocardiovascular pathology in pre-manifest and early-stage Huntington's disease.

BACKGROUND AND PURPOSE: Cardiovascular events are a major cause of early death in the Huntington's disease (HD) population. Dysautonomia as well as deterioration of circadian rhythms can be detected early in the disease progression and can have profound effects on cardiac health. The aim of the present study was to determine if patients with HD and pre-manifest mutation carriers present a higher risk of cardiovascular disease than non-mutation-carrying controls. METHODS: This was a prospective, cross-sectional, multicentre study of 38 HD mutation carriers (23 pre-manifest and 15 early-stage patients) compared with 38 age- and gender-matched healthy controls. Clinical and epidemiological variables, including the main haematological vascular risk factors, were recorded. Ambulatory blood-pressure monitoring and carotid intima-media thickness (CIMT) measurement were performed to assess autonomic function and as target-organ damage markers. RESULTS: Most (63.2%) patients with HD (86.7% and 47.8%, respectively, of the early-stage and pre-manifest patients) were non-dippers compared with 23.7% of controls (P = 0.001). CIMT values were in the 75th percentile in 46.7% and 43.5%, respectively, of the early-stage and pre-manifest patients, whereas none of the controls presented pathological values (P = 0.001 and P = 0.006, respectively). Nocturnal non-dipping was significantly associated with CIMT values in patients (P = 0.002) but not in controls. CONCLUSIONS: These results suggest that higher cardiovascular risks and target-organ damage are present even in pre-manifest patients. Although larger studies are needed to confirm these findings, clinicians should consider these results in the cardiovascular management of patients with HD.

[The hypothalamus in Huntington's disease]. / El hipotalamo en la enfermedad de Huntington.

INTRODUCTION: Disorders affecting sleep and the circadian rhythm, autonomic clinical signs and symptoms, and neuroendocrine alterations are frequent characteristics in Huntington's disease, some of which present in early stages of the disease. It is reasonable to think that some of these features could result from a hypothalamic dysfunction affecting the centre regulating sleep, metabolism and the autonomic nervous system. AIM: The study presents the evidence available to date that suggests the involvement of a hypothalamic disorder in Huntington's disease. DEVELOPMENT: Histopathological, hormonal and neuroimaging research relates this area of the brain to Huntington's disease. The experimental findings and those obtained with animal models or in studies conducted with patients are summarised. Likewise, the clinical repercussions (sleep and circadian rhythm disorders, psychiatric and cognitive pathologies, and the clinical signs and symptoms linked to autonomic dysfunction) secondary to possible involvement of the hypothalamus in this disease are also described. CONCLUSIONS: The hypothalamus acts as a centre that integrates the neuroendocrine and autonomic functions, and plays a significant role in cognitive and behavioural signs and symptoms. Disorders of this type have been highlighted in Huntington's disease. Further studies are needed to elucidate the role and scope of this region of the brain in this disease.

TITLE: El hipotalamo en la enfermedad de Huntington.Introduccion. Los trastornos del sueño y del ritmo circadiano, la sintomatologia autonomica y las alteraciones neuroendocrinas son caracteristicas frecuentes de la enfermedad de Huntington, y algunos de ellos se presentan en estadios tempranos de ella. Es plausible pensar que algunos de estos rasgos podrian ser el resultado de una disfuncion hipotalamica del centro regulador del sueño, el metabolismo y el sistema nervioso autonomo. Objetivo. Se exponen las evidencias disponibles hasta el momento actual que sugieren una afectacion hipotalamica en la enfermedad de Huntington. Desarrollo. Investigaciones histopatologicas, hormonales y de neuroimagen relacionan esta region cerebral con la enfermedad de Huntington. Se resumen los hallazgos, tanto experimentales como en modelos animales, o en estudios realizados con pacientes. Asimismo, se describen las repercusiones clinicas (alteraciones del sueño y del ritmo circadiano, la patologia psiquiatrica y cognitiva, y la clinica vinculada con disfuncion autonomica) secundarias a una posible afectacion hipotalamica en esta enfermedad. Conclusiones. El hipotalamo se comporta como un centro integrador de las funciones neuroendocrinas y autonomicas, y presenta un papel no desdeñable sobre la sintomatologia cognitiva y conductual. Alteraciones al respecto se han destacado en la enfermedad de Huntington. Son necesarios posteriores estudios para aclarar el papel y el alcance de esta region cerebral en esta enfermedad.

Relación entre el trastorno de conducta del sueño REM y el trastorno de control de impulsos en pacientes con enfermedad de Parkinson / Association between REM sleep behaviour disorder and impulse control disorder in patients with Parkinson's disease

Introducción: La relación entre el trastorno del control de impulsos (TCI) y el trastorno de conducta del sueño REM (TCSR) no se ha aclarado todavía y los resultados de la literatura son contradictorios. Nuestro objetivo es valorar la asociación entre estos 2 trastornos y, a su vez, su presencia en dependencia de la terapia dopaminérgica. Métodos: Un total de 73 pacientes diagnosticados de enfermedad de Parkinson, en tratamiento con un único agonista dopaminérgico, fueron incluidos en el estudio, tras valoración clínica y habiendo completado el cuestionario de pregunta única para el TCSR y el cuestionario abreviado para los trastornos impulsivo-compulsivos en la enfermedad de Parkinson. Resultados: La edad media ± desviación estándar de los pacientes fue de 68,88±7,758 años. De ellos, 26 pacientes (35,6%) se clasificaron dentro de un TCSR-probable, presentando mayor prevalencia de síndrome rígido acinético no tremórico, más años de tratamiento con levodopa y con agonistas dopaminérgicos, y una relación significativa con el TCI (p=0,001) en comparación con el grupo sin TCSR-probable. En cuanto al tratamiento con agonistas dopaminérgicos, se demostró la asociación significativa de la administración por vía oral con una mayor prevalencia de TCI, mientras que esta vía también se relacionó con mayor tendencia a desarrollar TCSR, diferencias en este caso no significativas. Conclusiones: Nuestros datos confirman que el TCSR se relaciona con el TCI en la enfermedad de Parkinson (AU)

Introduction: The relationship between impulse control disorder (ICD) and REM sleep behaviour disorder (RBD) has not yet been clarified, and the literature reports contradictory results. Our purpose is to analyse the association between these 2 disorders and their presence in patients under dopaminergic treatment. Methods: A total of 73 patients diagnosed with Parkinson's disease and treated with a single dopamine agonist were included in the study after undergoing clinical assessment and completing the single-question screen for REM sleep behaviour disorder and the short version of the questionnaire for impulsive-compulsive behaviours in Parkinson's disease. Results: Mean age was 68.88 ± 7.758 years. Twenty-six patients (35.6%) were classified as probable-RBD. This group showed a significant association with ICD (P=.001) and had a higher prevalence of non-tremor akinetic rigid syndrome and longer duration of treatment with levodopa and dopamine agonists than the group without probable-RBD. We found a significant correlation between the use of oral dopamine agonists and ICD. Likewise, patients treated with oral dopamine agonists demonstrated a greater tendency toward presenting probable-RBD than patients taking dopamine agonists by other routes; the difference was non-significant. Conclusions: The present study confirms the association between RBD and a higher risk of developing symptoms of ICD in Parkinson's disease (AU)