Brain imaging factors associated with progression of subcortical hyperintensities in CADASIL over 2-year follow-up.

BACKGROUND AND PURPOSE: Mutations in the NOTCH3 gene cause cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), a cerebral small vessel disease manifesting with stroke, migraine and dementia in adults. The disease displays significant phenotypic variability that is incompletely explained. Early abnormalities in vascular function have been shown in animal models. We postulated that studying changes in vascular function may offer insights into disease progression. METHODS: Twenty-two subjects with CADASIL [50% female, 50 (±11) years] from 19 pedigrees were included in a longitudinal multimodality study using brain magnetic resonance imaging (MRI), clinical measures, neuropsychology and measures of peripheral vascular function. MRI studies included measurement of structural brain changes, cerebral blood flow (CBF) and cerebrovascular reactivity by arterial spin labelling and a CO2 respiratory challenge. RESULTS: Over 2 years, new stroke or transient ischaemic attack (TIA) occurred in five (23%) subjects and new significant disability in one (5%). There were significant increases in number of lacunes, subcortical hyperintensity volume and microbleeds, and a decrease in brain volume. CBF declined by 3.2 (±4.5) ml/100 g/min over 2 years. CBF and carotid-femoral pulse wave velocity at baseline predicted change in subcortical hyperintensity volume at follow-up. Carotid intima-media thickness and age predicted brain atrophy. Baseline CBF was lower in subjects who showed a decline in attention and working memory. CONCLUSIONS: Cerebral blood flow predicts radiological progression of hyperintensities and thus is a potential biomarker of disease progression in CADASIL. Over 2 years, there were changes in several relevant imaging biomarkers (CBF, brain volume, lacunes, microbleeds and hyperintensity volume). Future studies in CADASIL should consider assessment of CBF as prognostic factor.

[Patients' perceptions of and attitudes towards epilepsy surgery: mistaken concepts in Colombia]. / Percepciones y actitudes de los pacientes frente a la cirugia de la epilepsia: conceptos equivocados en Colombia.

INTRODUCTION: Selected patients with drug-resistant focal epilepsy benefit from epilepsy surgery, however significant delays remain. The aim of this study was to assess knowledge and attitudes toward epilepsy surgery among patients with epilepsy and identify barriers that might delay the treatment. PATIENTS AND METHODS: A 10-minute questionnaire was administered to patients with epilepsy in Colombia. Survey assessed the following: knowledge of surgical options, perceptions about the risks of surgery vs. ongoing seizures, disease disability, treatment goals, and demographic and socioeconomic variables. RESULTS: We recruited 88 patients with focal epilepsy. More than half of patients (56%) were not aware that surgery might be an option. Apprehension about epilepsy surgery was evident, 60% of patients perceived epilepsy surgery to be very or moderately dangerous. A large proportion of patients believe death (41%), stroke (47%), vision loss (56%), personality change (56%), paralysis (62%), difficulties in speaking (69%), and memory loss (60%) were frequent side effects. The majority of patients (62%) consider the surgical procedure as the last option of treatment. CONCLUSIONS: There is a negative attitude toward epilepsy surgery based on the patients' misperceptions of suffering neurological deficits during the surgery, reflecting lack of knowledge toward this type of treatment. These perceptions can contribute to delays in surgical care.

TITLE: Percepciones y actitudes de los pacientes frente a la cirugia de la epilepsia: conceptos equivocados en Colombia.Introduccion. Los pacientes con epilepsia focal farmacorresistente se podrian beneficiar de la cirugia de la epilepsia; sin embargo, existen demoras en la realizacion del procedimiento. El objetivo de este estudio fue evaluar el conocimiento y las actitudes de los pacientes hacia la cirugia de epilepsia e identificar barreras que pudieran retrasar el tratamiento. Pacientes y metodos. Se aplico un cuestionario de 10 minutos a pacientes con epilepsia en Colombia. La encuesta evaluo el conocimiento de la opcion quirurgica, las percepciones sobre el riesgo de la cirugia frente al riesgo de crisis no controladas, la discapacidad producida por la enfermedad, las metas del tratamiento y las variables demograficas y socioeconomicas. Resultados. Se seleccionaron 88 pacientes con epilepsia focal. El 56% de los pacientes no sabia que la cirugia podria ser una opcion terapeutica. El 60% considero que la cirugia de la epilepsia es muy o moderadamente peligrosa. Una gran proporcion pensaba que la muerte (41%), el ictus (47%), la perdida visual (56%), los cambios en la personalidad (56%), la paralisis (61%), las dificultades para hablar (69%) y la perdida de la memoria (60%) eran efectos secundarios comunes. La mayoria (62%) consideraba el procedimiento como la ultima opcion de tratamiento. Conclusiones. Existe una actitud negativa por parte de los pacientes frente a la cirugia de la epilepsia fundamentada en la sobreestimacion del riesgo de adquirir deficits neurologicos secundarios al procedimiento, lo que refleja la falta de conocimiento hacia este tratamiento. Estas percepciones erroneas pueden contribuir a demoras en la atencion quirurgica.

Conocimientos y actitudes de los pacientes críticos y sus familiares respecto a las directivas anticipadas y la toma de decisiones al final de la vida / Knowledge and attitudes of critically ill patients and their families on advance directives and the decision making process at the end of life

La mayoría de los pacientes ingresados en las Unidades de Cuidados Intensivos presentan alteración de su estado de consciencia habitual siendo sus familiares o allegados los que ejercen como sustitutos. Resulta importante conocer los conocimientos y actitudes que adoptan los pacientes y sus representantes respecto al proceso de toma de decisiones al final de la vida. Objetivos: Explorar los conocimientos y las actitudes del paciente crítico y sus familiares respecto a las directivas anticipadas y conocer la forma de participación de la familia en la toma de decisiones al final de la vida. Método: Se realizó una revisión integrada en nueve bases de datos, con una combinación DeCs y MeSH, limitando la búsqueda a once años. La selección se realizó sobre documentos relevantes que trataban de los conocimientos y actitudes del paciente crítico y de sus familiares sobre las directivas anticipadas, así como del proceso de toma de decisiones. La lectura crítica se realizó mediante listas CASPE. Resultados: Se seleccionaron veinte artículos, de los cuales ocho son estudios descriptivos, cinco son analíticos y siete son estudios realizados con metodología cualitativa. En ellos se encuentra que tanto los conocimientos como el índice de otorgamientos de las directivas anticipadas (DA) son bajos y que la toma de decisiones de los familiares resulta un factor estresante que se ve aliviado por la existencia de DA y la comunicación entre profesionales sanitarios y familiares. Conclusiones: Las DA son poco conocidas, tanto por los pacientes como por sus familiares, aunque ambos muestran interés por conocer este instrumento que puede ayudar en la toma de decisiones al final de la vida. Los profesionales sanitarios también ven las DA junto con la comunicación, como buenas herramientas para la toma de decisiones

Most patients admitted to intensive care units have alterations in their usual state of consciousness, with the family or relatives serving as substitutes. It is important to determine the knowledge, as well as the attitudes adopted by patients and their representatives as regards the decision-making process at the end of life. Objectives: To determine and examine the knowledge and attitudes of critically ill patients and their families about advance directives and know how family involvement in decision making at the end of life. Method: A review was conducted using 9 integrated databases with a combination DeCs and MeSH, limiting the search to 11 years. Relevant documents that dealt with knowledge and attitudes of critically ill patients and their families about advance directives and the decision-making process were selected. Critical reading was performed using CASPE lists. Results: Twenty articles were selected, of which 8 were descriptive studies, 5 analytical, and 7 were studies performed using qualitative methodology. In these articles, it was found that both the knowledge and the rate of signing of advance directives (AD) are low, and that the decisions of the family is a stress factor that is alleviated by the existence of AD, as well as communication between health professionals and family. Conclusions: AD are poorly understood by both patients and their relatives, although both showed interest in learning about this tool that can help in decision-making at the end of life. Health professionals also see the AD, along with good communication, as tools for decision making

Variability in the frequency of rheumatology consultations in patients with rheumatoid arthritis in Spain.

The aim of this work was to assess the variability in the use of health-care resources, based on the number of visits to rheumatology departments by rheumatoid arthritis patients, in Spain, and its association with patient, physician and center characteristics. The sample consisted of patient records of men and women (aged 16 or older), with a clinical diagnosis of RA, who met the American Rheumatism Association 1987 revised criteria and who had been treated in a rheumatology department at a Spanish hospital with at least one visit to a rheumatologist during the two years preceding the date of the study. To analyze which variables were independently associated with the number of consultations, those with a statistically significant result in the bivariate analysis, or which were clinically relevant or deemed confounders, were used in the construction of a linear regression model. The records of 1188 RA patients were studied. The linear regression model explained the 26.67 % of the variability in the number of visits. The number of csDMARDs prescribed, the administration of biological therapy, corticoid prescription, the presence of nursing consultation, mean time to first visit in the department and attended population showed a positive significant association, while the presence of telephone consultation, distance from the hospital to the patient´s residence (≥20 km) and drug monitoring by rheumatology department + primary care physician or by other specialists were negatively associated with the number of consultations. We observed a high variability in the number of visits, which remains partially unexplained even after taking into account individual, physician and center characteristics.

Epidemiología, formas de presentación, estadio radiológico y métodos diagnósticos de la sarcoidosis en el área de León (2001-2008) / Epidemiology, presentation forms, radiological stage and diagnostic methods of sarcoidosis in the area of Leon (2001-2008)

Antecedentes. Los estudios epidemiológicos de la sarcoidosis en España y otros países son de hace más de una década. Objetivos. Conocer la incidencia actual en el área sanitaria de León, los motivos de consulta, la afectación radiológica torácica, el retraso y los métodos diagnósticos. Pacientes y métodos. Revisamos los casos de sarcoidosis atendidos en nuestro hospital entre los años 2001-2008. El estudio es retrospectivo. Resultados. Ciento dieciocho pacientes cumplieron criterios de sarcoidosis con una incidencia de 4,51/100.000 hab/año. Cincuenta y tres (44,9%) fueron varones con una media de edad de 36,4±12,2 y 65 (55,1%) mujeres, con edad media de 42,6±17,2 años (p=0,029). La forma de presentación más frecuente fue el síndrome de Löfgren, 45,8%; seguido de síntomas respiratorios, 20,3%; hallazgo radiológico, 12,7%; síndrome general, 10,2% y miscelánea 11%. La afectación radiológica torácica en el momento del diagnóstico: estadio I, 59,3%; estadio II, 19,8%; estadio III, 10%; fibrosis, 3,4%, y estadio 0, 8,5%. La biopsia transbronquial fue el medio diagnóstico más frecuente. El tiempo hasta el diagnóstico fue muy variable con una mediana de 24,5 días, 10 días cuando debutaba como eritema nudoso, 80 en las formas menos frecuentes. Conclusiones. La incidencia de sarcoidosis fue superior a la demostrada previamente. Afectó a adultos jóvenes pero con un aumento de los casos en mayores de 50 años con claro predominio femenino. El síndrome de Löfgren fue la forma de presentación más frecuente con mayor incidencia primaveral. El retraso diagnóstico varió según las diferentes formas de presentación. La biopsia transbronquial fue la principal herramienta diagnóstica(AU)

Backgrounds. The epidemiological studies of sarcoidosis in Spain and other countries are from more than one decade ago. Objectives. To know the current incidence in the health care area of Leon, the reasons for consultation, the thoracic radiologyl involvement, delay and diagnostic methods. Patients and methods. We reviewed the cases of sarcoidosis seen in our hospital between the years 2001-2008. The study is retrospective. Results. 118 patients fulfilled the criteria for sarcoidosis with an incidence of 4.51/100,000 inhab./year. Of these, 53 (44.9%) were male with a mean age of 36.4±12.2 and 65 (55.1%) women, with a mean age of 42.6±17.2 years (P=0.029). The most frequent presentation form was Löfgren Syndrome with 45.8%, followed by respiratory symptoms 20.3%, radiological findings 12.7%, general syndrome 10.2% and miscellaneous 11%. Thoracic radiological affectations at the time of diagnosis were: stage I: 59.3%, stage II: 19.8%, stage III: 10%, fibrosis 3.4% and stage 0: 8.5%. Transbronchial biopsy was the most frequent diagnosis. Time to diagnosis was very variable with a median of 24.5 days, when it debuted as erythema nodosum, 80 in the less frequent forms. Conclusions. The incidence of sarcoidosis was superior to that previously demonstrated. It affected young adults, but with an increase in the number of cases in those over 50 years with clear female predominance. Löfgren syndrome was the most frequent presentation form, with a higher incidence in spring. Diagnostic delay varied according to the different presentation forms. Transbronchial biopsy was the main diagnostic tool(AU)

[Epidemiology, presentation forms, radiological stage and diagnostic methods of sarcoidosis in the area of Leon (2001-2008)]. / Epidemiología, formas de presentación, estadio radiológico y métodos diagnósticos de la sarcoidosis en el área de León (2001-2008).

BACKGROUNDS: The epidemiological studies of sarcoidosis in Spain and other countries are from more than one decade ago. OBJECTIVES: To know the current incidence in the health care area of Leon, the reasons for consultation, the thoracic radiologyl involvement, delay and diagnostic methods. PATIENTS AND METHODS: We reviewed the cases of sarcoidosis seen in our hospital between the years 2001-2008. The study is retrospective. RESULTS: 118 patients fulfilled the criteria for sarcoidosis with an incidence of 4.51/100,000 inhab./year. Of these, 53 (44.9%) were male with a mean age of 36.4 ± 12.2 and 65 (55.1%) women, with a mean age of 42.6 ± 17.2 years (P = 0.029). The most frequent presentation form was Löfgren Syndrome with 45.8%, followed by respiratory symptoms 20.3%, radiological findings 12.7%, general syndrome 10.2% and miscellaneous 11%. Thoracic radiological affectations at the time of diagnosis were: stage I: 59.3%, stage II: 19.8%, stage III: 10%, fibrosis 3.4% and stage 0: 8.5%. Transbronchial biopsy was the most frequent diagnosis. Time to diagnosis was very variable with a median of 24.5 days, when it debuted as erythema nodosum, 80 in the less frequent forms. CONCLUSIONS: The incidence of sarcoidosis was superior to that previously demonstrated. It affected young adults, but with an increase in the number of cases in those over 50 years with clear female predominance. Löfgren syndrome was the most frequent presentation form, with a higher incidence in spring. Diagnostic delay varied according to the different presentation forms. Transbronchial biopsy was the main diagnostic tool.

Anisakiasis intestinal diagnosticada por enteroclisis / Intestinal anisakiasis diagnosed by enteroclysis

La anisakiasis es una parasitación frecuente en nuestro medio y que da lugar a cuadros de dolor abdominal que pueden simular otras patologías, incluyendo situaciones quirúrgicas (apendicitis o peritonitis), pero generalmente autolimitadas. Presentamos un caso de una paciente de 48 años con dolor abdominal agudo, febrícula y hallazgos patológicos en TAC (propios de un cuadro inflamatorio intestinal), en la que la realización de una enteroclisis fue capaz de identificar larvas de Anisakis. La paciente evolucionó favorablemente con tratamiento conservador y el patrón radiológico volvió a la normalidad. En nuestro caso, la posibilidad de contar con una técnica radiológica objetiva que sirvió de ayuda al diagnóstico proporcionó datos muy útiles en el manejo de la paciente. Se revisan las características clínicas y radiológicas de la enfermedad (AU)

Anisakiasis is a common infestation in our setting that produces abdominal pain that can simulate other diseases including surgical conditions (symptoms of acute abdomen, mimicking appendicitis or peritonitis), but it is generally a self-limiting process. We report a case of a 48-year-old female with acute abdominal pain, fever and pathological findings on the CT scan (inflammatory bow condition). Anisakis larvae could be identified by the enteroclysis. The patient evolved favorably with conservative treatment and radiologic intestinal pattern returned to normal. The possibility of a radiological technical to assist in the diagnosis provided very useful data in the management of our patient. The radiological and clinical features of the disease are reviewed (AU)

Treatment of refractory posterior uveitis with infliximab: a 7-year follow-up study.

OBJECTIVE: To describe, in a 7-year follow-up study, the use of infliximab in patients with refractory posterior uveitis and scleritis. METHODS: A 7-year follow-up case series study was performed. Patients with posterior uveitis and scleritis refractory to conventional therapies (steroids and at least one immunosuppressive agent) were included. Three infliximab intravenous doses of 5 mg/kg were administered at weeks 0, 2, and 6. Further infusions were allowed in patients undergoing a relapse of the uveitis after initial remission. All patients were followed up for at least 8 months. We defined uveitis improvement as an increase in the best-corrected visual acuity or an objective and significant improvement in retinal exudates and/or haemorrhages, cystoid macular oedema (CME), and vitreous haze. Infliximab-related adverse events, final prednisone doses, and the number of immunosuppressive agents used were recorded. A descriptive analysis was performed. RESULTS: A total of 11 patients (17 eyes were affected at baseline) were included, 63% were women, the mean age was 43+/-14 years, and the median follow-up was 80 months (p25-p75: 50-80). After infliximab treatment, six eyes maintained their basal visual acuity, nine eyes showed improvement, and two worsened (in the two patients diagnosed with choroiditis). Vitreous haze, active retinal vasculitis, and CME, but not chorioretinal lesions, improved in all patients. All patients tapered their daily steroid dose and the number of immunosuppressive agents. No infliximab-related adverse events were reported. CONCLUSIONS: Infliximab could be an effective and safe treatment in patients with posterior uveitis and scleritis refractory to conventional therapy.

Differences between rheumatology attending physicians and training residents in the management of rheumatoid arthritis in Spain.

OBJECTIVE: To evaluate the variability in the characteristics and management of rheumatoid arthritis (RA) patients between rheumatology attending physicians and training residents in Spain. METHODS: A retrospective medical record (MR) review was performed in a probabilistic sample of 1379 RA patients from 46 centres distributed in 16 of the 19 autonomous communities (AC) of Spain. RA patients' sociodemographic and clinical characteristics, healthcare resources use, and their single responsible physician's (defined as an identifiable single physician who attended the patient in more than 75% of visits) characteristics were recorded following a standardized protocol. Multivariate analyses were performed to assess differences in the characteristics and management of RA patients between attending physicians and training residents. RESULTS: A total of 1205 RA patients had a single responsible physician and were analysed (nearly 75% women with rheumatoid factor positive and more than 25% with persistent active disease), 49 of whom were followed by training residents and 1156 by attending physicians. In the multivariate analyses, irrespective of patient and disease characteristics, training residents' patients reported more hospital admissions, laboratory tests, and imaging techniques compared to attending physicians. Training residents also less frequently used combined therapy with disease-modifying antirheumatic drugs (DMARDs). CONCLUSION: Training residents and attending physicians differ in RA patients' care. More efforts in training programmes are necessary to guarantee proper RA management and to improve the profile of the future rheumatologists.

Síndrome de Melkersson-Rosenthal, a propósito de un caso con parálisis facial bilateral / Melkersson-Rosenthal syndrome, about one case with bilateral facial paralysis

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Upregulation of the macrophage scavenger receptor in response to different forms of injury in the CNS.

The monoclonal antibody 2F8 was used to localize the macrophage scavenger receptor by immunohistochemistry. In control adult mice, macrophage scavenger receptor expression in the brain was restricted to stromal and epiplexus macrophages of the choroid plexus, meningeal macrophages and to perivascular sites. Microglia did not express the receptor. In the developing mouse brain, macrophage scavenger receptor expression was high on meningeal macrophages and detectable on immature microglia in the supraventricular corpus callosum, cingulum, cavum septum and the periaqueductal area. In the aged mouse brain, the pattern of macrophage scavenger receptor expression was no different from that in the young adult brain. Macrophage scavenger receptor expression on resident microglia and recruited macrophages was detected 24 h after an intrahippocampal injection of either lipopolysaccharide or kainic acid. Macrophage scavenger receptor expression was also detected in microglia 3 days after optic nerve crush both in the nerve segment distal to the crush site and in the superior colliculus. These studies indicate a potential role for the macrophage scavenger receptor in the CNS in the clearance of debris during acute neuronal degeneration.

[Renal angiomyolipoma: a cause of massive retroperitoneal hemorrhage]. / Angiomiolipoma renal: una causa de hemorragia retroperitoneal masiva.

Five patients were diagnosed as having angiomyolipoma. Evidence or signs of tuberous sclerosis were observed in no patient. In 2 patients the presenting symptoms was massive retroperitoneal hemorrhage which required emergency surgery. The kidney could be preserved in 4 cases. One underwent nephrectomy for synchronous clear cell renal tumor. CT and US afford a diagnostic accuracy of nearly 100 percent. According to the data reported in the literature, tumors larger than 4 cm are amenable to treatment by conservative surgery. Smaller tumors can be followed carefully. However, surgical exploration is warranted not only in patients with pain and hemorrhage, but also in those cases with complex lesions less than 4 cm.