Gut dysbiosis in cutaneous T-cell lymphoma is characterized by shifts in relative abundances of specific bacterial taxa and decreased diversity in more advanced disease.

BACKGROUND: Cutaneous T-cell lymphoma (CTCL) patients often suffer from recurrent skin infections and profound immune dysregulation in advanced disease. The gut microbiome has been recognized to influence cancers and cutaneous conditions; however, it has not yet been studied in CTCL. OBJECTIVES: To investigate the gut microbiome in patients with CTCL and in healthy controls. METHODS: A case-control study was conducted between January 2019 and November 2020 at Northwestern's busy multidisciplinary CTCL clinic (Chicago, Illinois, USA) utilizing 16S ribosomal RNA gene amplicon sequencing and bioinformatics analyses to characterize the microbiota present in fecal samples of CTCL patients (n = 38) and age-matched healthy controls (n = 13) from the same geographical region. RESULTS: Gut microbial α-diversity trended lower in patients with CTCL and was significantly lower in patients with advanced CTCL relative to controls (P = 0.015). No differences in ß-diversity were identified. Specific taxa were significantly reduced in patient samples; significance was determined using adjusted P-values (q-values) that accounted for a false discovery rate threshold of 0.05. Significantly reduced taxa in patient samples included the phylum Actinobacteria (q = 0.0002), classes Coriobacteriia (q = 0.002) and Actinobacteria (q = 0.03), order Coriobacteriales (q = 0.003), and genus Anaerotruncus (q = 0.01). The families Eggerthellaceae (q = 0.0007) and Lactobacillaceae (q = 0.02) were significantly reduced in patients with high skin disease burden. CONCLUSIONS: Gut dysbiosis can be seen in patients with CTCL compared to healthy controls and is pronounced in more advanced CTCL. The taxonomic shifts associated with CTCL are similar to those previously reported in atopic dermatitis and opposite those of psoriasis, suggesting microbial parallels to the immune profile and skin barrier differences between these conditions. These findings may suggest new microbial disease biomarkers and reveal a new angle for intervention.

Preoperative giant sacrococcygeal teratoma embolization in a newborn - A case report and a review. / Embolización preoperatoria de teratoma sacrococcigéo gigante en un recién nacido. Reporte de un caso y revisión.

Sacrococcygeal teratoma (SCT) is the most frequent congenital germ cell tumor. Patients have a higher risk of perinatal complications and death, with bleeding and cardiac decompensation being the most common causes of neonatal mortality. This is the case of a 35-week preterm newborn with a large SCT diagnosed at ultrasound screening in the second trimester. Preoperative selective embolization of the middle sacral artery and total surgical resection were performed postnatally with minimal blood loss. The patient was discharged at 25 days of life with a normal physical examination. Selective embolization prior to giant SCT resection is feasible and appears as a safe and useful technique in the control of perioperative bleeding.

El teratoma sacrococcígeo (TSC) es el tumor congénito de células germinales más frecuente. Los pacientes afectados tienen un mayor riesgo de complicaciones perinatales y muerte, siendo la hemorragia y la descompensación cardiaca las causas más comunes de mortalidad neonatal. Presentamos el caso de un recién nacido pretérmino de 35 semanas con un TSC de gran tamaño diagnosticado por ecografía en el segundo trimestre. La embolización selectiva preoperatoria de la arteria sacra media y la resección quirúrgica total postnatal se realizaron con una mínima pérdida de sangre. El paciente fue dado de alta a los 25 días de vida con un examen físico normal. La embolización selectiva antes de la cirugía de resección del TSC gigante es factible y aparece como una técnica segura y útil en el control del sangrado perioperatorio.

Embolización preoperatoria de teratoma sacrococcígeo gigante en un recién nacido. Reporte de un caso y revisión / Preoperative giant sacrococcygeal teratoma embolization in a newborn - A case report and a review

El teratoma sacrococcígeo (TSC) es el tumor congénito de células germinales más frecuente. Los pacientes afectados tienen un mayor riesgo de complicaciones perinatales y muerte, siendo la hemorragia y la descompensación cardiaca las causas más comunes de mortalidad neonatal. Presentamos el caso de un recién nacido pretérmino de 35 semanas con un TSC de gran tamaño diagnosticado por ecografía en el segundo trimestre. La embolización selectiva preoperatoria de la arteria sacra media y la resección quirúrgica total postnatal se realizaron con una mínima pérdida de sangre. El paciente fue dado de alta a los 25 días de vida con un examen físico normal. La embolización selectiva antes de la cirugía de resección del TSC gigante es factible y aparece como una técnica segura y útil en el control del sangrado perioperatorio

Sacrococcygeal teratoma (SCT) is the most frequent congenital germ cell tumor. Patients have a higher risk of perinatal complications and death, with bleeding and cardiac decompensation being the most common causes of neonatal mortality.This is the case of a 35-week preterm newborn with a large SCT diagnosed at ultrasound screening in the second trimester. Preoperative selective embolization of the middle sacral artery and total surgical resection were performed postnatally with minimal blood loss. The patient was discharged at 25 days of life with a normal physical examination. Selective embolization prior to giant SCT resection is feasible and appears as a safe and useful technique in the control of perioperative bleeding

Global patterns of care in advanced stage mycosis fungoides/Sezary syndrome: a multicenter retrospective follow-up study from the Cutaneous Lymphoma International Consortium.

BACKGROUND: Advanced-stage mycosis fungoides (MF)/Sézary syndrome (SS) patients are weighted by an unfavorable prognosis and share an unmet clinical need of effective treatments. International guidelines are available detailing treatment options for the different stages but without recommending treatments in any particular order due to lack of comparative trials. The aims of this second CLIC study were to retrospectively analyze the pattern of care worldwide for advanced-stage MF/SS patients, the distribution of treatments according to geographical areas (USA versus non-USA), and whether the heterogeneity of approaches has potential impact on survival. PATIENTS AND METHODS: This study included 853 patients from 21 specialist centers (14 European, 4 USA, 1 each Australian, Brazilian, and Japanese). RESULTS: Heterogeneity of treatment approaches was found, with up to 24 different modalities or combinations used as first-line and 36% of patients receiving four or more treatments. Stage IIB disease was most frequently treated by total-skin-electron-beam radiotherapy, bexarotene and gemcitabine; erythrodermic and SS patients by extracorporeal photochemotherapy, and stage IVA2 by polychemotherapy. Significant differences were found between USA and non-USA centers, with bexarotene, photopheresis and histone deacetylase inhibitors most frequently prescribed for first-line treatment in USA while phototherapy, interferon, chlorambucil and gemcitabine in non-USA centers. These differences did not significantly impact on survival. However, when considering death and therapy change as competing risk events and the impact of first treatment line on both events, both monochemotherapy (SHR = 2.07) and polychemotherapy (SHR = 1.69) showed elevated relative risks. CONCLUSION: This large multicenter retrospective study shows that there exist a large treatment heterogeneity in advanced MF/SS and differences between USA and non-USA centers but these were not related to survival, while our data reveal that chemotherapy as first treatment is associated with a higher risk of death and/or change of therapy and thus other therapeutic options should be preferable as first treatment approach.

Ablación percutánea mediante radiofrecuencia de tumores renales en pacientes de alto riesgo: 10 años de experiencia / Percutaneous radiofrequency ablation of renal tumors in high-risk patients: 10 years’ experience

Objetivo. Evaluar retrospectivamente la eficacia y la seguridad de la radiofrecuencia (RF) percutánea para el tratamiento de tumores renales en pacientes de alto riesgo o con riesgo de padecer tumores renales múltiples con un seguimiento a medio plazo a lo largo de un periodo de 10 años. Material y métodos. Entre 2005 y 2015, 87 pacientes (89 tumores) con una media de edad de 73,7 años (desviación estándar: 10,87) con riesgo quirúrgico alto que presentaban tumores renales T1a y T1b fueron tratados mediante RF. Se excluyeron los pacientes sometidos a RF y embolización o ablación con microondas. Se trataron carcinomas de células claras (43,6%), papilares (17,2%), cromófobos (10,3%), tumores quísticos (2,2%) y un angiomiolipoma (1,1%). El tamaño medio de los tumores fue 2,6cm. La tomografía computarizada y/o la ecografía se utilizaron para guiar el procedimiento. Se analizó la relación entre la eficacia y la edad de los pacientes, el tipo de aguja, el origen de los pacientes, el tamaño y localización tumoral, y el número de sesiones necesarias para ablacionar el tumor. Se registraron las complicaciones. Resultados. La RF se pudo realizar a todos los pacientes. La media de seguimiento fue de 32,1 meses. La eficacia fue de un 93,7%. El 87,5% de los pacientes requirieron una sola sesión, el 8%, 2 sesiones y el 4,5%, 3 sesiones. El único factor asociado a una menor eficacia fue el tamaño del tumor (p=0,03). La tasa de complicaciones fue del 5,6%. Conclusiones. La RF es eficaz y segura, con resultados comparables a los referidos en la literatura especializada (AU)

Objective. To retrospectively evaluate the efficacy and safety of percutaneous radiofrequency ablation (RFA) done to treat renal tumors in patients with high surgical risk or with the risk of developing multiple renal tumors in the medium term at our center over a period of 10 years. Material and methods. Between 2005 and 2015, we used RFA to treat 89 T1a or T1b tumors in 87 patients (mean age, 73.7±10.87 years) with high surgical risk. We excluded patients treated with radiofrequency and embolization or microwave ablation. The tumors treated were clear cell carcinomas (43.6%), papillary renal carcinomas (17.2%), chromophobe renal cell carcinomas (10.3%), cystic tumors (2.2%), and an angiomyolipoma (1.1%). The mean size of the tumors was 2.6cm. Computed tomography and/or ultrasonography were used to guide the procedure. We analyzed the relation between the efficacy of the procedure and patients’ age, the type of needle, the source of the patients, the size and location of the tumor, and the number of sessions required to achieve ablation. We recorded all complications. Results. The RFA procedure was completed in all patients. The mean follow-up period was 32.1 months. The efficacy was 93.7%. A single session was sufficient in 87.5% of patients; 8% required two sessions and 4.5% required three sessions. The only factor associated with worse efficacy was the size of the tumor (p=0.03). The rate of complications was 5.6%. Conclusions. RFA is efficacious and safe, with results comparable to those reported in the literature (AU)

Percutaneous radiofrequency ablation of renal tumors in high-risk patients: 10 years' experience. / Ablación percutánea mediante radiofrecuencia de tumores renales en pacientes de alto riesgo: 10 años de experiencia.

OBJECTIVE: To retrospectively evaluate the efficacy and safety of percutaneous radiofrequency ablation (RFA) done to treat renal tumors in patients with high surgical risk or with the risk of developing multiple renal tumors in the medium term at our center over a period of 10 years. MATERIAL AND METHODS: Between 2005 and 2015, we used RFA to treat 89 T1a or T1b tumors in 87 patients (mean age, 73.7±10.87 years) with high surgical risk. We excluded patients treated with radiofrequency and embolization or microwave ablation. The tumors treated were clear cell carcinomas (43.6%), papillary renal carcinomas (17.2%), chromophobe renal cell carcinomas (10.3%), cystic tumors (2.2%), and an angiomyolipoma (1.1%). The mean size of the tumors was 2.6cm. Computed tomography and/or ultrasonography were used to guide the procedure. We analyzed the relation between the efficacy of the procedure and patients' age, the type of needle, the source of the patients, the size and location of the tumor, and the number of sessions required to achieve ablation. We recorded all complications. RESULTS: The RFA procedure was completed in all patients. The mean follow-up period was 32.1 months. The efficacy was 93.7%. A single session was sufficient in 87.5% of patients; 8% required two sessions and 4.5% required three sessions. The only factor associated with worse efficacy was the size of the tumor (p=0.03). The rate of complications was 5.6%. CONCLUSIONS: RFA is efficacious and safe, with results comparable to those reported in the literature.

Merkel cell carcinoma: a review.

Merkel cell carcinoma (MCC) is a rare aggressive primary cutaneous carcinoma with high mortality and rising incidence. The exact etiology of MCC remains unclear, but it is likely multifactorial with many factors playing a role, among these, ultraviolet radiation, immunosuppression, and recently, Merkel cell polyomavirus. Clinically MCC appears as an asymptomatic, firm, skin colored, sometimes reddish-blue, dome-shaped papule or plaque or subcutaneous nodule typically localized on the head and neck region that has grown rapidly. As its clinical presentation is generally non specific, the diagnosis relies on histological and immunohistochemical findings. Once diagnosis is established, adequate staging requires evaluation of regional and distant metastases. Treatment is based on multidisciplinary management although optimal therapy is controversial, at least in part due to a lack of quality data. Aggressive surgery frequently associated with adjuvant radiotherapy is used to improve the rates of locoregional recurrence and overall survival as well. Future targeted therapies may open new perspectives for the treatment of patients although high-quality, multicentre and randomized studies are needed. In this article, the current knowledge about MCC is reviewed and discussed.

γδ T-cell-rich variants of pityriasis lichenoides and lymphomatoid papulosis: benign cutaneous disorders to be distinguished from aggressive cutaneous γδ T-cell lymphomas.

BACKGROUND: T cells with a γδ phenotype have been associated with aggressive lymphomas. Yet, inflammatory skin disorders and low-grade lymphoproliferative disorders have rarely been described with a predominant γδ T-cell infiltrate. OBJECTIVES: To review our experience and determine the clinical relevance of the γδ T-cell phenotype in lymphomatoid papulosis (LyP) and pityriasis lichenoides (PL). METHODS: A retrospective dermatopathology file review looking for LyP and PL characterized by a γδ T-cell phenotype was performed. Clinical manifestations and course, histological features and molecular data were analyzed. RESULTS: Six of 16 cases of LyP and four of 23 cases diagnosed as PL during a 5-year period (2009-14) were identified. The median follow-up for the whole group was 16 months (range 3-64), showing an indolent clinical course in all cases. CONCLUSIONS: The detection of a predominantly γδ T-cell phenotype in papular lymphoid-rich infiltrates in the absence of other lesions is not associated with a clinically aggressive course. γδ T-cell-rich variants of LyP and PL may reflect a spectrum of related conditions. This is a single academic centre retrospective chart review of a relatively small sample.

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

[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.

[Prevention of migraine: a pharmacoepidemiological study]. / Prevención de la migraña: un estudio farmacoepidemiológico.

INTRODUCTION: This study aims to perform a descriptive analysis of the usage patterns of migraine prophylactic medications by various neurologists in our setting. MATERIAL AND METHODS: The first preventive treatment prescribed for migraine in patients not associated to other diagnoses of primary headache was recorded in three outpatient neurology clinics and one headache specific clinic. RESULTS: A total of 235 prophylactic treatments out of 669 patients were initiated. The patients were aged 37 +/- 12 years (mean +/- standard deviation) and 84.45% were women. Migraines with aura accounted for 18.9% of migraines. By order of frequency, the prophylactic treatments administered were topiramate (43%), beta-blockers (18%), flunaricine (17%), amitriptyline (14%), selective serotonin reputake inhibitors (6%) and others (2%). Beta-blockers and flunaricine were used much more frequently in men (29.7% and 27% versus 15.9% and 14.4%, respectively) and antidepressants were used more in women (21.87% versus 5.4%). The most frequently used antidepressant was amitriptyline, and its use increases with the age of the patient, it being the most frequently used treatment in over 60-year-old patient group. CONCLUSIONS: At present, topiramate has become the first preventive treatment option for migraine in our setting, especially in young women. There is greater variability in the choice of an alternative treatment. Amitriptyline is the first choice within the antidepressants and is almost exclusively prescribed in women with migraine and elderly age.

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)

Prevención de la migraña: un estudio farmacoepidemiológico / Prevention of migraine: a pharamacoepidemiological study

Introducción. Se realiza un análisis descriptivo de los tratamientosprofilácticos empleados para el tratamiento de la migrañapor varios neurólogos de nuestro medio.Material y métodos. Se registró el primer tratamiento preventivopautado para migraña en pacientes que no asociaban otro diagnósticode cefalea primaria. Se reclutaron durante 1 año en tres consultasde neurología ambulatoria y una específica de cefalea de laprovincia de Alicante.Resultados. De 669 pacientes totales, se iniciaron 235 tratamientosprofilácticos, con una edad de los pacientes de 37±12años (media±desviación estándar) y un 84,45 % de mujeres. Un18,9% de migrañas eran con aura. Por orden de frecuencia, los profilácticosempleados fueron el topiramato (43 %), los betabloqueantes(18%), la flunaricina (17%), la amitriptilina (14 %), inhibidoresselectivos de la recaptación de serotonina (6 %) y otros(2 %). Los betabloqueantes y la flunaricina se emplearon muchomás en el hombre (29,7 y 27 % frente a 15,9 y 14,4%, respectivamente)y los antidepresivos en la mujer (21,87 frente a 5,4 %). Elantidepresivo más empleado fue la amitriptilina, y su empleo aumentacon la edad del paciente, siendo el tratamiento más frecuenteen el grupo de más de 60 años.Conclusiones. El topiramato en la actualidad y en nuestro medioes la primera opción en el tratamiento preventivo de la migraña,sobre todo en la mujer joven. La variabilidad es mayor en la eleccióndel tratamiento alternativo. La amitriptilina es la primera opcióndentro de los antidepresivos y se prescriben casi exclusivamente en lamujer con migraña y en edades avanzadas (AU)

Introduction. This study aims to perform a descriptiveanalysis of the usage patterns of migraine prophylactic medicationsby various neurologists in our setting.Material and methods. The first preventive treatment prescribedfor migraine in patients not associated to other diagnosesof primary headache was recorded in three outpatient neurologyclinics and one headache specific clinic.Results. A total of 235 prophylactic treatments out of 669patients were initiated. The patients were aged 37±12 years(mean±standard desviation) and 84.45% were women. Migraineswith aura accounted for 18.9% of migraines. By order of frequency,the prophylactic treatments administered were topiramate(43%), beta-blockers (18%), flunaricine (17%), amitriptyline(14%), selective serotonin reputake inhibitors (6 %) and others(2 %). Beta-blockers and flunaricine were used much more frequentlyin men (29.7% and 27% versus 15.9% and 14.4%, respectively)and antidepressants were used more in women(21.87% versus 5.4%). The most frequently used antidepressantwas amitriptyline, and its use increases with the age of the patient,it being the most frequently used treatment in over 60 yearold patient group.Conclusions. At present, topiramate has become the firstpreventive treatment option for migraine in our setting, especiallyin young women. There is greater variability in the choiceof an alternative treatment. Amitriptyline is the first choice withinthe antidepressants and is almost exclusively prescribed inwomen with migraine and elderly age (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