A peptide derived from phage-display limits psoriasis-like lesions in mice.

BACKGROUND: Psoriasis is a pro-inflammatory disease with unknown etiology, that is characterized by skin inflammation and keratinocytes hyperproliferation. Specific inhibition of inflammation has shown positive effects avoiding the progression of the psoriatic lesions in different animal models of the disease, turning this strategy as a remarkable therapeutic alternative. OBJECTIVE: To screen the effectiveness of a novel IFN-α/ß signalling inhibitor in the development reduction of skin lesions in IMQ and TPA mice models of psoriasis. METHODS: We used a Phage-peptide library for the screening of a peptide with inhibitory effects on the development of psoriasis-like lesions in mice. To evaluate the in vivo effect of the phage-peptides (Phpep3D) and the derived peptide (Pep3D), we administered Phpep3D or Pep3D intradermally in mice with imiquimod (IMQ)-induced psoriasis and 12-O-tetradecanoyl phorbol-13-acetate (TPA)-induced psoriasis. We scored the lesions, and we determined the number of neutrophils and the production of some pro-inflammatory cytokines in the lesions. RESULTS: In this work, we describe how the Ph3pepD and Pep3D reduced skin thickness, redness, and acanthosis despite the presence of the psoriasis inducers, IMQ or TPA. We also found that Pep3D reduced the number of GR1+ infiltrated cells and decreased the production of IL-17A and TNFα in the psoriatic skin of mice. In-silico, docking analysis showed that Pep3D may interact with the interferon-alpha receptor, but further analyses should be performed to uncover the mechanism of action of this peptide. CONCLUSION: Our results suggest that Pep3D could be used as a new treatment for psoriasis.

Antimicrobial activity of an aspartic protease from Salpichroa origanifolia fruits.

Plant proteases play a fundamental role in several processes like growth, development and in response to biotic and abiotic stress. In particular, aspartic proteases (AP) are expressed in different plant organs and have antimicrobial activity. Previously, we purified an AP from Salpichroa origanifolia fruits called salpichroin. The aim of this work was to determine the cytotoxic activity of this enzyme on selected plant and human pathogens. For this purpose, the growth of the selected pathogens was analysed after exposure to different concentrations of salpichroin. The results showed that the enzyme was capable of inhibiting Fusarium solani and Staphylococcus aureus in a dose-dependent manner. It was determined that 1·2 µmol l-1 of salpichroin was necessary to inhibit 50% of conidial germination, and the minimal bactericidal concentration was between 1·9 and 2·5 µmol l-1 . Using SYTOX Green dye we were able to demonstrate that salpichroin cause membrane permeabilization. Moreover, the enzyme treated with its specific inhibitor pepstatin A did not lose its antibacterial activity. This finding demonstrates that the cytotoxic activity of salpichroin is due to the alteration of the cell plasma membrane barrier but not due to its proteolytic activity. Antimicrobial activity of the AP could represent a potential alternative for the control of pathogens that affect humans or crops of economic interest. SIGNIFICANCE AND IMPACT OF THE STUDY: This study provides insights into the antimicrobial activity of an aspartic protease isolated from Salpichroa origanifolia fruits on plant and human pathogens. The proteinase inhibited Fusarium solani and Staphylococcus aureus in a dose-dependent manner due to the alteration of the cell plasma membrane barrier but not due to its proteolytic activity. Antimicrobial activity of salpichroin suggests its potential applications as an important tool for the control of pathogenic micro-organisms affecting humans and crops of economic interest. Therefore, it would represent a new alternative to avoid the problems of environmental pollution and antimicrobial resistance.

Is entheses ultrasound reliable? A reading Latin American exercise.

The objective of this study is to evaluate inter-reader entheses ultrasound (US) reliability and the influence of the type of image or degree of sonographer experience on US reliability in patients with spondyloarthritis (SpA). Eighteen Latin American ultrasonographers with different experience took part in an US reading exercise evaluating 60 entheseal images (50 % static images and 50 % videos) from healthy controls and SpA patients. The following sonographic lesions were assessed: structure, thickness, bone proliferation/tendon calcification, erosions, bursitis, and Doppler signal. Another group of three experts with significant experience in entheses US read all images too. Inter-reader reliability among participants and experts was calculated by the Cohen's kappa coefficient. Thresholds for kappa values were <0.2 poor, 0.21-0.4 fair, 0.41-0.6 moderate, 0.61-0.8 good, and 0.81-1 excellent. Furthermore, the results for the expert group were stratified based on the type of image. Kappa correlation coefficients among participants, showed variability depending on the type of lesion, being fair for structure and thickness, moderate for calcifications, erosions, and bursitis, and excellent for Doppler signal. Inter-reader reliability among experts was higher, being moderate for structure and thickness, good for calcifications and bursitis, and excellent for erosions and Doppler. Inter-reader reliability for assessing calcification and structure using static images was significantly higher than for videos. Overall inter-reader reliability for assessing entheses by US in SpA is moderate to excellent for most of the lesions. However, special training seems fundamental to achieve better inter-reader reliability. Moreover, the type of image influenced these results, where evaluation of entheses by videos was more difficult than by static images.

Cuerpo extraño en el paladar de bebé / Foreign body in the palate of a baby

Se presenta el caso de un paciente de sexo femenino de 10 meses de edad referida a la Clínica Dental de la Universidad Cayetano Heredia por presentar una tumoración en el paladar, con un tiempo de enfermedad de 2 meses. Fue examinada por un odontólogo particular el cual le prescribió antibióticos y la derivo a la clínica dental Cayetano Heredia. La evaluó el departamento de cirugía bucomaxilofacial y ellos la derivaron a Odontopediatria. AI examen clínico intraoral se observe una tumoración amarillenta en forma de "frejol" en la línea media del paladar dura rodeado de un rodete de mucosa de coloración normal. El diagnostico presuntivo fue lipoma. Se hizo una interconsulta con Medicina y Patología Oral, llegándose al diagnóstico definitivo de: "objeto extraño", procediéndose procedió al retiro seguido de la limpieza de la zona. Se dieron indicaciones de cuidados a los padres...

This paper reports the case of an 8 months baby girl who was derived to UPCH Dental Clinic. She presented a tumor on the palate for 2 months. First, she was evaluated by a general dentist who prescribed her antibiotics and derived her to UP CH Dental Clinic. She was evaluated by the department of Bucomaxillofacial Surgery who derived the patient to Pediatric Dentistry. By the clinical examination it was noticed a yellow tumor which has bean form in the middle of the hard palate rounded by normal oral mucosa. The initial diagnosis was lipoma. An interconsultation was done with Medicine and Oral Pathology. The definitive diagnosis was foreign body which was taken out. The zone behind it was cleaned and her parents were instructed about how to do it...

[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.

Comparison of Microcystis aeruginosa (PCC7820 and PCC7806) growth and intracellular microcystins content determined by liquid chromatography-mass spectrometry, enzyme-linked immunosorbent assay anti-Adda and phosphatase bioassay.

Cyanobacteria are able to produce several metabolites that have toxic effects on humans and animals. Among these cyanotoxins, the hepatotoxic microcystins (MC) occur frequently. The intracellular MC content produced by two strains of Microcystis aeruginosa, PCC7806 and PCC7820, and its production kinetics during the culture time were studied in order to elucidate the conditions that favour the growth and proliferation of these toxic strains. Intracellular MC concentrations measured by liquid chromatography (LC) coupled to electrospray ionization mass spectrometer (MS) were compared with those obtained by enzyme-linked immunosorbent assay (ELISA) anti-Adda and protein phosphatase 2A (PP2A) inhibition assays. It has been demonstrated there are discrepancies in the quantification of MC content when comparing ELISA and LC-MS results. However, a good correlation has been obtained between PP2A inhibition assay and LC-MS. Three MC were identified using LC-MS in the PCC7806 strain: MC-LR, demethylated MC-LR and a new variant detected for the first time in this strain, [L-MeSer(7)] MC-LR. In PCC7820, MC-LR, D-Asp(3)-MCLR, Dglu(OCH3)-MCLR, MC-LY, MC-LW and MC-LF were identificated. The major one was MC-LR in both strains, representing 81 and 79% of total MC, respectively. The total MC content in M. aeruginosa PCC7820 was almost three-fold higher than in PCC7806 extracts.

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.

High NaCl concentrations induce the nod genes of Rhizobium tropici CIAT899 in the absence of flavonoid inducers.

The nodulation (nod) genes of Rhizobium tropici CIAT899 can be induced by very low concentrations (micromolar to nanomolar range) of several flavonoid molecules secreted by the roots of leguminous plants under a number of different conditions. Some of these conditions have been investigated and appear to have a great influence on the concentration and the number of different Nod factors, which can induce root nodule primordia and pseudonodules in several leguminous plant roots. In one such condition, we added up to 300 mM NaCl to the induction medium of R. tropici CIAT899 containing the nod gene inducer apigenin. At the higher concentrations of NaCl, larger amounts and more different Nod factors were produced than in the absence of extra NaCl. To our surprise, under control conditions (300 mM NaCl without apigenin), some Nod-factor-like spots were also observed on the thin-layer plates used to detect incorporation of radiolabeled glucosamine into newly synthesized Nod factors. This phenomenon was further investigated with thin-layer plates, fusions of nod genes to the lacZ gene, high-performance liquid chromatography, mass spectrometry, and the formation of pseudonodules on bean roots. Here, we report that, in the absence of flavonoid inducers, high concentrations of NaCl induced nod genes and the production of Nod factors.

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