[Migraine with prolonged eyelid edema: a series of 10 cases]. / Migraña con edema palpebral prolongado: serie de 10 casos.

INTRODUCTION: Migraine may present with cranial autonomic symptoms typical of trigeminal-autonomic cephalalgias, thus posing diagnostic difficulties. AIM. To report a series of patients with prominent eyelid oedema associated with migraine. PATIENTS AND METHODS: Ten patients attending the headache offices in three hospitals (nine women, one man; age: 26-53 years-old) with recurrent eyelid oedema as a migraine accompaniment. RESULTS: According to the diagnostic criteria of the International Classification of Headache Disorders (ICHD-III, beta version), eight patients had migraine without aura, one had migraine with aura, and one had chronic migraine. Eyelid oedema appeared during the most severe headache attacks, and had longer duration than the pain. Pharmacological or systemic causes of the oedema were ruled out in all cases. Other associated autonomic symptoms were conjunctival injection (n = 3), lacrimation (n = 2) and rhinorrhoea (n = 1). Both the pain and the oedema improved with symptomatic and preventive therapies for migraine. CONCLUSIONS: Eyelid oedema may occasionally be a migraine accompaniment. It appears in some patients during their most severe migraine attacks, and may improve with the acute and preventive treatment for migraine.

TITLE: Migraña con edema palpebral prolongado: serie de 10 casos.Introduccion. La migraña puede cursar con sintomas autonomicos craneales propios de las cefaleas trigeminoautonomicas, lo que plantea dificultades en el diagnostico. Objetivo. Describir una serie de diez pacientes con edema palpebral asociado a la migraña. Pacientes y metodos. Diez pacientes atendidos en la consulta de cefaleas de tres hospitales (nueve mujeres, un varon; edad: 26-53 años), con edema palpebral recurrente asociado a la migraña. Resultados. Segun los criterios diagnosticos de la Clasificacion Internacional de las Cefaleas (ICHD-III, version beta), ocho pacientes presentaban migraña sin aura, una tenia migraña con aura y otra, migraña cronica. El edema palpebral aparecia durante las crisis de migraña mas intensas, y tenia mayor duracion que la cefalea. Se descartaron causas farmacologicas o sistemicas del edema en todos los casos. Otros sintomas autonomicos asociados fueron la inyeccion conjuntival (n = 3), el lagrimeo (n = 2) y la rinorrea (n = 1). Tanto el dolor como el edema asociado respondieron a los tratamientos sintomaticos y preventivos de la migraña. Conclusiones. El edema palpebral es un posible acompañante de la migraña. Aparece en algunos pacientes con los episodios de mayor intensidad, y responde al tratamiento sintomatico y preventivo de la migraña.

Red de cuidadores informales de los pacientes con demencia en la provincia de Alicante, descripción de sus características / Characteristics of informal caregivers of patients with dementia in Alicante province

Introducción: Los cuidadores informales garantizan el cuidado de los pacientes con demencia, prolongando la estancia en su domicilio. Objetivos: Describir las características de los pacientes con demencia de la provincia de Alicante, así como los perfiles y roles de los cuidadores implicados en su manejo. Pacientes y métodos: Estudio prospectivo multicéntrico realizado en 4 consultas de neurología de la provincia de Alicante (junio del 2009-enero del 2010). Inclusión consecutiva de familiares/cuidadores de pacientes con demencia. Analizamos: a) datos demográficos del paciente y cuidadores (edad/sexo, estado civil/laboral, estudios, parentesco); b) unidad de convivencia del paciente; c) motivación para el cuidado del cuidador principal (CP); d) roles del cuidador secundario (CS); e) nacionalidad del cuidador formal (CF) y origen remuneración (privada/institucional), y f) formación en demencias de los cuidadores. Resultados: Nuestros pacientes residen en su domicilio (74,8%). Principalmente, son mujeres (69%) con enfermedad de Alzheimer (78,4%) moderadamente grave (GDS 4-5, 71,6%). CP y CS son mujeres (72,1% vs 60,5%), de edad media, parentesco directo (hijo/a 64,3% vs 54,4%), nivel cultural bajo y amas de casa. Las primeras garantizan el cuidado por obligación moral (75%), las segundas se ocupan del ocio/estimulación del paciente (82,3%). Los grandes ausentes son los varones (73,3%), quienes residen lejos del familiar (52,4%). El CF es una mujer (91,7%), espa˜nola (81,8%) con remuneración privada. Conclusiones: La mujer es el elemento principal en la red de cuidadores de pacientes con demencia: como CP, de apoyo o CF (todos con escasa formación en demencias), siendo el varón el gran ausente. Conocer la estructura de cuidados de estos pacientes facilitará su manejo (AU)

Introduction: Informal caregivers provide care to dementia patients, and this service prolongs their stay at home. Objectives: To describe characteristics of dementia patients in the province of Alicante, as well as the profiles and roles of caregivers who assist them. Patients and methods: Multi-centre prospective study carried out in 4 neurology departments in Alicante (June 2009 to January 2010). Dementia patients’ relatives/caregivers were included in sequential order. The following variables were analysed: a) Demographic information pertaining to the patient and caregivers (age, sex, marital and employment status, educational level, relationship to patient); b) patient’s family unit; c) motivating factor for primary caregiver (PC); d) secondary caregiver (SC) roles; e) country of citizenship of formal caregiver (FC) and source of remuneration (private/public); f) caregivers’ knowledge of dementia. Results: Most of our patients live at home (74.8%), and are female (69%) with Alzheimer’s disease (78.4%) in a moderately severe stage (GDS level 4-5, 71.6%). PCs and SCs are mainly women (72.1% and 60.5% respectively), middle-aged and directly related to the patient (sons/daughters account for 64.3% of the PCs and 54.4% of the SCs); most are homemakers with a low educational level. Caregivers in the first category (PC) provide care due to moral obligation (75%), while those in the second (SC) involve patients in leisure or other stimulating activities (82.3%). Absent caregivers tend to be males (73.3%) residing long distances from the relative (52.4%). The FC tends to be female (91.7%), Spanish (81.8%) and privately remunerated. Conclusions: Women dominate the network of caregivers for dementia patients, whether as principal caregivers, supporting caregivers or formal caregivers (in all cases, they have only limited training in dementia management). Males are largely absent. Better knowledge of the care structure supporting dementia patients may be helpful in the overall management of these patients (AU)

Characteristics of informal caregivers of patients with dementia in Alicante province.

INTRODUCTION: Informal caregivers provide care to dementia patients, and this service prolongs their stay at home. OBJECTIVES: To describe characteristics of dementia patients in the province of Alicante, as well as the profiles and roles of caregivers who assist them. PATIENTS AND METHODS: Multi-centre prospective study carried out in 4 neurology departments in Alicante (June 2009 to January 2010). Dementia patients' relatives/caregivers were included in sequential order. The following variables were analysed: a) Demographic information pertaining to the patient and caregivers (age, sex, marital and employment status, educational level, relationship to patient); b) patient's family unit; c) motivating factor for primary caregiver (PC); d) secondary caregiver (SC) roles; e) country of citizenship of formal caregiver (FC) and source of remuneration (private/public); f) caregivers' knowledge of dementia. RESULTS: Most of our patients live at home (74.8%), and are female (69%) with Alzheimer's disease (78.4%) in a moderately severe stage (GDS level 4-5, 71.6%). PCs and SCs are mainly women (72.1% and 60.5% respectively), middle-aged and directly related to the patient (sons/daughters account for 64.3% of the PCs and 54.4% of the SCs); most are homemakers with a low educational level. Caregivers in the first category (PC) provide care due to moral obligation (75%), while those in the second (SC) involve patients in leisure or other stimulating activities (82.3%). Absent caregivers tend to be males (73.3%) residing long distances from the relative (52.4%). The FC tends to be female (91.7%), Spanish (81.8%) and privately remunerated. CONCLUSIONS: Women dominate the network of caregivers for dementia patients, whether as principal caregivers, supporting caregivers or formal caregivers (in all cases, they have only limited training in dementia management). Males are largely absent. Better knowledge of the care structure supporting dementia patients may be helpful in the overall management of these patients.

Cefalea menstrual: subtipos, fenotipos clínicos y tratamiento / Menstrual headache: subtypes, clinical phenotypes and treatment

Introducción. Más del 60% de las mujeres refieren presentar cefalea relacionada con el ciclo menstrual. Se ha incluido la ‘migraña menstrual’ en la segunda edición de la Clasificación Internacional de las Cefaleas; sin embargo, la ‘cefaleatensional durante la menstruación’ es una entidad no reconocida por la Sociedad Internacional de Cefaleas.Objetivos. Evaluar la prevalencia de los diferentes subtipos de cefalea menstrual y analizar sus características clínicas y eltratamiento prescrito.Pacientes y métodos. Evaluamos de forma prospectiva a todas las mujeres atendidas en varias consultas de neurología,desde enero a noviembre de 2008, cuya cefalea aparecía durante el período menstrual. Resultados. Se incluyeron un total de 108 pacientes durante el período del estudio, con una edad media de 34,8 ± 8,9 años. El 29,3% presentaba migraña menstrual pura, el 58,7% migraña menstrual relacionada con la menstruación, el 4,5% cefalea tensional pura durante la menstruación y el 7,5% cefalea tensional relacionada con la menstruación. Conclusiones. Nuestros resultados sugieren que la cefalea tensional en el contexto de la menstruación existe, con unaprevalencia en torno al 12% en las pacientes valoradas en nuestras consultas (AU)

Introduction. Up to 60% of women relate their episodes of headache to menstrual cycle. Menstrual migraine has been included in the second edition of the International Classification of Headache disorders. Menstrual tension-type headache has not yet been recognised by the International Headache Society. Aims. To evaluate the prevalence of different subtypes of menstrual headache and to analyze their clinical features and the treatment prescribed. Patients and methods. We prospectively included women attending several neurology outpatient clinics, from January toNovember 2008 whose headache appeared during the menstrual period. Results. A total of 108 patients were included during the study period. Mean age was 34.8 ± 8.9 years-old. 29.3% sufferedfrom pure menstrual migraine, 58.7% from menstrual related migraine, 4.5% from pure menstrual tension-type headacheand 7.5% from menstrual related tension type headache.Conclusions. Our data suggest that menstrual related tension-type headaches exist with a prevalence found about 12%, inour neurology outpatient clinics

Dos nuevos casos de enfermedad de Creutzfeldt-Jakob esporádica en Madrid, España / Two new cases of sporadic Creutzfeldt-Jakob disease in Madrid, Spain

Introducción. La enfermedad de Creutzfeldt-Jakob es una encefalopatía espongiforme transmisible, en la que típicamente se produce el acúmulo de una isoforma alterada de la proteína priónica en el cerebro. La forma esporádica se caracteriza por presentar una demencia rápidamente progresiva, con síntomas y signos de afectación piramidal, extrapiramidal, cerebelosa, cortical y presencia de mioclonías. El diagnóstico se basa en los hallazgos clínicos en combinación con los resultados típicos del electroencefalograma, estudio de líquido cefalorraquídeo o la neuroimagen. Casos clínicos. Presentamos dos casos detectados entre los años 2008 y 2010 en el Hospital del Henares, en Coslada. Caso 1: mujer con pérdida de fuerza progresiva en las extremidades, cambio en su comportamiento con labilidad emotiva y tendencia al aislamiento. Valorada inicialmente como una paciente psiquiátrica, en los meses posteriores presentó una situación de inmovilidad absoluta con tendencia al mutismo. El fallecimiento se produjo a los seis meses del inicio de los síntomas. Caso 2: varón con cuadro progresivo de alteración de la visión en forma de hemianopsia homónima derecha, dificultad para la emisión del lenguaje, mareo e inestabilidad. Progresivamente manifestó un síndrome cerebeloso con mioclonías generalizadas hasta su fallecimiento, 10 semanas después del inicio de los síntomas. En ambos casos el electroencefalograma mostró un patrón típico y la proteína 14-3-3 fue positiva en el estudio de líquido cefalorraquídeo. Conclusión. Nos ha llamado la atención una incidencia tan elevada en tan corto período y en un área sanitaria de apenas 170.000 habitantes (AU)

Introduction. Creutzfeldt-Jakob disease is a transmissible spongiform encephalopathy, in which there is typically an accumulation of an altered isoform of the prion protein in the brain. The sporadic form is characterised by presenting a rapidly progressive dementia, with signs and symptoms of pyramidal, extrapyramidal, cerebellar and cortical involvement, as well as the presence of myoclonias. Diagnosis is based on the clinical findings in combination with the typical results of an electroencephalogram, cerebrospinal fluid study or neuroimaging. Case reports. We report two cases detected between the years 2008 and 2010 in the Hospital del Henares, in Coslada, Madrid. Case 1: a female with progressive loss of strength in her limbs, change in her behaviour with mood swings and a tendency towards seeking to isolate herself. Although initially considered a psychiatric patient, in the months that followed she became absolutely immobile with a tendency towards mutism. Death occurred six months after the onset of symptoms. Case 2: a male with a progressive picture of altered vision in the form of right homonymous hemianopia, difficulty in verbally expressing language, dizziness and instability. The patient gradually developed a cerebellar syndrome with generalised myoclonias until his death, 10 weeks after the onset of symptoms. In both cases, the electroencephalogram showed a typical pattern and 14-3-3 protein was positive in the cerebrospinal fluid study. Conclusions. Such a high incidence over such a short period of time and in a health district of barely 170,000 inhabitants seems rather surprising (AU)

[Two new cases of sporadic Creutzfeldt-Jakob disease in Madrid, Spain]. / Dos nuevos casos de enfermedad de Creutzfeldt-Jakob esporadica en Madrid, Espana.

INTRODUCTION: Creutzfeldt-Jakob disease is a transmissible spongiform encephalopathy, in which there is typically an accumulation of an altered isoform of the prion protein in the brain. The sporadic form is characterised by presenting a rapidly progressive dementia, with signs and symptoms of pyramidal, extrapyramidal, cerebellar and cortical involvement, as well as the presence of myoclonias. Diagnosis is based on the clinical findings in combination with the typical results of an electroencephalogram, cerebrospinal fluid study or neuroimaging. CASE REPORTS: We report two cases detected between the years 2008 and 2010 in the Hospital del Henares, in Coslada, Madrid. Case 1: a female with progressive loss of strength in her limbs, change in her behaviour with mood swings and a tendency towards seeking to isolate herself. Although initially considered a psychiatric patient, in the months that followed she became absolutely immobile with a tendency towards mutism. Death occurred six months after the onset of symptoms. Case 2: a male with a progressive picture of altered vision in the form of right homonymous hemianopia, difficulty in verbally expressing language, dizziness and instability. The patient gradually developed a cerebellar syndrome with generalised myoclonias until his death, 10 weeks after the onset of symptoms. In both cases, the electroencephalogram showed a typical pattern and 14-3-3 protein was positive in the cerebrospinal fluid study. CONCLUSIONS: Such a high incidence over such a short period of time and in a health district of barely 170,000 inhabitants seems rather surprising.

[Telephone attendance system in a dementia unit: analysis and description]. / Descripcion y analisis del sistema de actividad telefonica implantado en una unidad de demencias.

AIM: To describe the characteristics of a telephone attendance system (TAS) implemented in our dementia unit, its relevance in the global activity and the users-themselves utility and satisfaction. MATERIALS AND METHODS: We retrospectively analyzed the incoming telephone calls during a period of one year. Telephone call type, user's gender and relationship with the patient, reason for calling, who answer, class of answer and patients' Global Deterioration Scale (GDS) were systematically registered. Caregivers' satisfaction was evaluated by a semi structured telephone questionnaire checking the following variables: accessibility, difficulty in answer comprehension, utility and global satisfaction. Professionals' opinion about global impression, overcharge in daily activity, TAS satisfaction and utility were evaluated by a semi structured interview. RESULTS: On the period of study, 444 telephone incoming calls were registered, 58.3% of them from relatives of patients with GDS 5-6. To adjust the treatment (43.2%) or to bring forward the next appointment were the most frequent responses. Next aspects were considered as good or very good for interviewed users: accessibility (58.3%), response delay (83.3%), service utility (91.6%), global satisfaction (86.5%) and answer comprehension (93.8%). Professionals' opinion were good, but with some comments about the need for a specific appointment book, improvements aspects in initial accessibility (direct telephone line), and the establishment of a specific schedule for telephonic attendance. CONCLUSIONS: The TAS is a formal, structured and complementary attendance service for traditional visit, with the ability to resolve most problems. The delayed reply mode allows the clinicians to choose the best moment to reply. Furthermore, the availability of the complete chart of every patient attended improve the quality of the clinical answer.

Descripción y análisis del sistema de actividad telefónica implantado en una unidad de demencias / Telephone attendance system in a dementia unit: analysis and description

Resumen. Objetivo. Describir el sistema de atención telefónica (SAT) implantado en una unidad de demencias y conocer la utilidad percibida y la satisfacción de los usuarios y profesionales implicados. Materiales y métodos. Analizamos retrospectivamente las llamadas recibidas durante 12 meses. Recogimos sistemáticamente: tipo de llamada, sexo y parentesco de quien llama, motivo, tipo de respuesta y puntuación del paciente en la escala de deterioro global (Global Deterioration Scale, GDS). Valoramos mediante cuestionario semiestructurado la satisfacción de pacientes/familiares con: accesibilidad, demora, dificultad en comprender la respuesta, utilidad y satisfacción global. Valoramos la opinión de los profesionales mediante entrevista semiestructurada para: impresión global, sobrecarga asistencial, utilidad y satisfacción del SAT. Resultados. Se registraron 44 llamadas, el 58,3% de familiares de pacientes con GDS 5-6. Las causas más frecuentes fueron síntomas psicoconductuales (32,6%) y efectos adversos de la medicación (22%). Las respuestas más frecuentes fueron modificar el tratamiento (43,2%) o adelantar la siguiente visita (18,9%). Los usuarios valoraron positivamente los siguientes conceptos: accesibilidad (58,3%), demora en la respuesta (83,3%), utilidad del servicio (91,6%), satisfacción global (86,5%) y comprensión de la respuesta (93,8%). Los profesionales señalaron la necesidad de confeccionar una agenda específica, mejorar la accesibilidad telefónica y programar tiempos específicos para la atención telefónica. Conclusiones. El SAT constituye un sistema de atención formal, estructurado y complementario a la visita tradicional, con capacidad para resolver los problemas planteados. Su carácter diferido permite gestionar los tiempos de atención y mejorar la calidad de la respuesta al disponer de toda la información del paciente. Es satisfactorio para usuarios y profesionales (AU)

Summary. Aim. To describe the characteristics of a telephone attendance system (TAS) implemented in our dementia unit, its relevance in the global activity and the users-themselves utility and satisfaction. Materials and methods. We retrospectively analyzed the incoming telephone calls during a period of one year. Telephone call type, user’s gender and relationship with the patient, reason for calling, who answer, class of answer and patients’ Global Deterioration Scale (GDS) were systematically registered. Caregivers’ satisfaction was evaluated by a semi structured telephone questionnaire checking the following variables: accessibility, difficulty in answer comprehension, utility and global satisfaction. Professionals’ opinion about global impression, overcharge in daily activity, TAS satisfaction and utility were evaluated by a semi structured interview. Results. On the period of study, 444 telephone incoming calls were registered, 58.3% of them from relatives of patients with GDS 5-6. To adjust the treatment (43.2%) or to bring forward the next appointment were the most frequent responses. Next aspects were considered as good or very good for interviewed users: accessibility (58.3%), response delay (83.3%), service utility (91.6%), global satisfaction (86.5%) and answer comprehension (93.8%). Professionals’ opinion were good, but with some comments about the need for a specific appointment book, improvements aspects in initial accessibility (direct telephone line), and the establishment of a specific schedule for telephonic attendance. Conclusions. The TAS is a formal, structured and complementary attendance service for traditional visit, with the ability to resolve most problems. The delayed reply mode allows the clinicians to choose the best moment to reply. Furthermore, the availability of the complete chart of every patient attended improve the quality of the clinical answer (AU)

[Clinical and biomolecular classification of the frontotemporal dementias. A review of the literature]. / Clasificación clínica y biomolecular de las demencias frontotemporales. Revisión de la bibliografía.

INTRODUCTION: The term 'frontotemporal lobar dementia' (FTLD) covers a group of neurodegenerative diseases that are very heterogeneous in their clinical expression, genetic component and histopathological features, and this has traditionally made it difficult to study and classify them. Patients usually present a progressive change in their behaviour associated with language disorders and loss of memory, which constitutes the second most important cause of dementia in persons under the age of 65. The most significant characteristic at the histopathological level is the presence of abnormal aggregates or accumulations of proteins in neurons or glial cells; their identification has, on the one hand, helped further our knowledge of the pathogenic mechanisms and, on the other hand, has allowed this type of dementia to be classified. DEVELOPMENT AND CONCLUSIONS: In the last two decades a remarkable amount of progress has been made in our knowledge of this group of diseases, thanks to the genetic advances related to the discovery of the MAPT gene and the progranulin gene, as well as their mutations, which are responsible for a high percentage of cases of hereditary FTLD. Likewise, the development of new immunohistochemical techniques has made it possible to characterise some abnormal proteins, such as the protein TDP-43, as the main component of the neuronal inclusions in tau-negative FTLD. All this has led to a new classification of the FTLD. This work includes a thorough review of said advances and the possible clinical, histological, genetic and biomolecular correlations of the different subtypes of FTLD are also considered.

Clasificación clínica y biomolecular de las demencias frontotemporales. Revisión de la bibliografía / Clinical and biomolecular classification of the frontotemporal dementias. A review of the literature

Introducción. El término ‘demencia lobar frontotemporal’ (DLFT) agrupa un conjunto de enfermedades neurodegenerativas muy heterogéneo en su expresión clínica, componente genético y características histopatológicas, lo que tradicionalmente ha dificultado su estudio y clasificación. Los pacientes presentan de forma habitual un cambio progresivo en su conducta asociado a alteración del lenguaje y pérdida de memoria, lo que constituye la segunda causa de demencia en personas menores de 65 años. La característica más relevante a nivel histopatológico es la presencia de agregados o acúmulos de proteínas anómalas en las neuronas o la glía, cuya identificación ha contribuido, por una parte, al conocimiento de los mecanismos patogénicos y, por otra, ha permitido la clasificación de este tipo de demencia. Desarrollo y conclusiones. En las dos últimas décadas, se ha producido un notable progreso en el conocimiento de este grupo de enfermedades, gracias a los avances genéticos relacionados con el descubrimiento del gen MAPT y el gen de la progranulina, así como de sus mutaciones, responsables de un porcentaje elevado de las DLFT hereditarias; igualmente, el desarrollo de nuevas técnicas inmunohistoquímicas ha permitido caracterizar algunas proteínas anómalas, como la proteína TDP-43, como principal integrante de las inclusiones neuronales en las DLFT tau negativas. Todo ello ha permitido establecer una nueva clasificación de las DLFT. En el presente trabajo se realiza una revisión exhaustiva de dichos avances, además de considerar las posibles correlaciones clínicas, histológicas, genéticas y biomoleculares de los distintos subtipos de DLFT

Introduction. The term ‘frontotemporal lobar dementia’ (FTLD) covers a group of neurodegenerative diseases thatare very heterogeneous in their clinical expression, genetic component and histopathological features, and this has traditionally made it difficult to study and classify them. Patients usually present a progressive change in their behaviour associated with language disorders and loss of memory, which constitutes the second most important cause of dementia inpersons under the age of 65. The most significant characteristic at the histopathological level is the presence of abnormal aggregates or accumulations of proteins in neurons or glial cells; their identification has, on the one hand, helped further ourknowledge of the pathogenic mechanisms and, on the other hand, has allowed this type of dementia to be classified.Development and conclusions. In the last two decades a remarkable amount of progress has been made in our knowledge of this group of diseases, thanks to the genetic advances related to the discovery of the MAPT gene and the progranulin gene, as well as their mutations, which are responsible for a high percentage of cases of hereditary FTLD. Likewise, the development of new immunohistochemical techniques has made it possible to characterise some abnormal proteins, such as the protein TDP-43, as the main component of the neuronal inclusions in tau-negative FTLD. All this has led to a new classification of the FTLD. This work includes a thorough review of said advances and the possible clinical, histological, genetic and biomolecularcorrelations of the different subtypes of FTLD are also considered

Parkinsonismo y deterioro cognitivo reversibles tras tratamiento con ácido valproico / Reversible Parkinsonism and cognitive impairment following treatment with valproate

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Encefalitis subaguda por el virus de la parotiditis en un adulto / No disponible

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