LAENALS: epidemiological and clinical features of amyotrophic lateral sclerosis in Latin America.

OBJECTIVE: The Latin American Epidemiologic study of ALS (LAENALS) aims to gather data on ALS epidemiology, phenotype, and risk factors in Cuba, Chile, and Uruguay, to understand the impact of genetic and environmental factors on ALS. METHODS: A harmonized data collection protocol was generated, and a Latin-American Spanish language Register was constructed. Patient data were collected in Uruguay in 2018, in Chile from 2017 to 2019, and in Cuba between 2017 and 2018. Statistical analysis was performed using SPSS 25.0.0 software. Crude cumulative incidence, standardized incidence, and prevalence were calculated in the population aged 15 years and older. RESULTS: During 2017-2019, 90 people with ALS from Uruguay (55.6% men), 219 from Chile (54.6% men), and 49 from Cuba (55.1% men) were included. The cumulative crude incidence in 2018 was 1.73/100,000 persons in Uruguay, 1.08 in Chile and 0.195 in Cuba. Crude prevalence in 2018 was 2.19 per 100,000 persons in Uruguay, 1.39 in Chile and 0.55 in Cuba. Mean age at onset was 61.8 ± 11.96 SD years in Uruguay, 61.9 ± 10.4 SD years in Chile, and 60.21 ± 12.45 SD years in Cuba (p = 0.75). Median survival from onset was 32.43 months (21.93 - 42.36) in Uruguay, 24 months (13.5 - 33.5) in Chile, and 29 months (15 - 42.5) in Cuba (p = 0.006). CONCLUSIONS: These preliminary data from LAENALS confirm the lower incidence and prevalence of ALS in counties with admixed populations. The LAENALS database is now open to other Latin American countries for harmonized prospective data collection.

Biomarcadores de estrés oxidativo en la enfermedad de Huntington y otros desórdenes neurológicos: estudio comparativo

Fundamento: los biomarcadores de estrés oxidativo en la enfermedad de Huntington pudieran predecir el curso de la enfermedad y evaluar nuevos tratamientos, pero su naturaleza inespecífica parece impedir la identificación de algún marcador útil. Esclarecer similitudes y diferencias de este fenómeno y su comportamiento con características clínicas puede ser esencial. Objetivo comparar biomarcadores de estrés oxidativo entre pacientes con enfermedad de Huntington y con otros desórdenes neurológicos. Métodos se realizó un estudio analítico, retrospectivo y caso-control (enfermedad de Huntington esclerosis lateral amiotrófica, ataxia espinocerebelosa tipo 2 e ictus isquémico: en etapa aguda y crónica). Se recogieron variables demográficas, clínicas y marcadores de daño oxidativo (malonildialdehído, productos avanzados de oxidación a proteínas) y antioxidantes (superóxido dismutasa: catalasa: glutatión peroxidasa, capacidad antioxidante del plasma). Resultados hubo diferencias significativas del malonildialdehído en la enfermedad de Huntington respecto al control (p=0,02), pero no con el resto de los grupos. La enzima superóxido dismutasa en enfermedad de Huntington fue menor estadísticamente en comparación con esclerosis lateral amiotrófica, aunque para la catalasa fue superior en relación con el resto de los pacientes. El FRAP en enfermedad de Huntington resultó menor significativamente versus esclerosis lateral amiotrófica e ictus isquémico agudo. Los productos avanzados de la oxidación de proteína se correlacionaron directamente con las edades biológicas y de inicio de la enfermedad de Huntington. La actividad motora en la esclerosis lateral amiotrófica y el déficit neurológico en el ictus isquémico agudo se correlacionaron con el malonildialdehído y glutatión peroxidasa respectivamente. Conclusiones la enfermedad de Huntington parece mostrar características específicas en su sistema antioxidante. La oxidación de proteínas pudiera estar relacionada con la acumulación de huntingtina mutada en el tiempo.

Foundation: biomarkers of oxidative stress in Huntington's disease could predict the course of the disease and evaluate new treatments, but their nonspecific nature seems to prevent the identification of any useful marker. Clarifying similarities and differences of this phenomenon and its behavior with clinical characteristics may be essential. Objective: compare biomarkers of oxidative stress between patients with Huntington's disease and other neurological disorders. Methods: an analytical, retrospective and case-control study was carried out (Huntington's disease, amyotrophic lateral sclerosis, spinocerebellar ataxia type 2 and ischemic stroke: acute and chronic stage). Demographic and clinical variables and markers of oxidative damage (malonildialdehyde, advanced protein oxidation products) and antioxidants (superoxide dismutase: catalase: glutathione peroxidase, plasma antioxidant capacity) were collected. Results: there were significant differences in malonyldialdehyde in Huntington's disease compared to the control (p=0.02), but not with the rest of the groups. The enzyme superoxide dismutase in Huntington's disease was statistically lower compared to amyotrophic lateral sclerosis, although for catalase it was higher in relation to the rest of the patients. FRAP in Huntington's disease was significantly lower versus amyotrophic lateral sclerosis and acute ischemic stroke. Advanced products of protein oxidation were directly correlated with the biological and onset ages of Huntington's disease. Motor activity in amyotrophic lateral sclerosis and neurological deficit in acute ischemic stroke were correlated with malonyldialdehyde and glutathione peroxidase, respectively. Conclusions: huntington's disease seems to show specific characteristics in its antioxidant system. Protein oxidation could be related to the accumulation of mutated huntingtin over time.

The Latin American Epidemiology Network for ALS (Laenals).

Background: There is evolving evidence of non-uniform distribution of ALS worldwide, with apparently lower incident and prevalent rates outside populations of European origin. However, the phenotype, survival and environmental risk in populations of mixed ancestral origin have not been well established. Large scale population based studies of incidence, prevalence, phenotype and risk factors in admixed populations are necessary to determine the true demography of ALS, and to test the hypothesis of differential risk and phenotype in populations of mixed ancestry. Methods: The Latin American Epidemiological Network of ALS (LAENALS) has been established to perform a comparative analysis of ALS epidemiology between three different Latin American populations (Cuba, Uruguay and Chile), and to test the hypothesis that the demographics, phenotype and outcome of ALS are influenced by ancestral origin, and that environmental and occupational risk factors differ across different ethnicities due to subtle differences in gene- environmental interactions. Recognition and interrogation of these differences is an important step toward novel therapeutic approaches and personalized medicine for all ALS both in the US, and worldwide. Discussion: This work will enable direct and detailed comparative studies between different ancestral populations with varying degrees of admixture, with facility for comparison with a large European reference dataset for ALS, and will provide a unique and rich dataset of admixed populations for later comparative genomic studies.

Automedicación en pacientes mayores de 60 con enfermedades neurológicas / Self-Medication in Patients Older than Sixty Years and with Neurologic Diseases

Introducción: La automedicación es un fenómeno creciente y ha sido considerado un problema de salud pública en la actualidad. Objetivo: Estimar el nivel de automedicación relacionado con el retraso diagnóstico de pacientes con enfermedades neurológicas y los factores asociados a esta táctica de autocuidado. Métodos: Se proyectó un estudio observacional de corte transversal, en el que se incluyeron 102 pacientes y 100 familiares pertenecientes al municipio Plaza de la Revolución, atendidos en consulta de Neurología del Instituto de Neurología y Neurocirugía, con diagnóstico de enfermedades neurológicas, durante el período de un año (junio 2018-junio2019). Resultados: Del total de pacientes, 45,1 por ciento manifestó automedicarse, fue más frecuente en mujeres (63,1 por ciento), con predominio en pacientes no universitarios (90 por ciento) y amas de casa (54,3 por ciento). El síntoma principal que motivó la automedicación fue la cefalea (30,4 por ciento), y los analgésicos no opioides fue el grupo de mayor consumo (23,9 por ciento). La latencia al diagnóstico de enfermedades neurológicas en el grupo de automedicados fue entre 10 a 19 meses mayor que en los no automedicados. Conclusiones: La automedicación constituye una práctica muy frecuente, lo cual produjo el retraso diagnóstico y terapéutico de diversas enfermedades neurológicas de baja incidencia, dando al traste con una evolución adecuada de ellas(AU)

Introduction: Self-medication is a growing phenomenon and, today, has been considered a public health concern. Objective: To estimate the level of self-medication related to the diagnostic delay of patients with neurological diseases and the factors associated with this self-care tactic. Methods: An observational and cross-sectional study was designed, in including 102 patients and one hundred relatives from Plaza de la Revolución Municipality, who received attention, during a one-year period (June 2018-June 2019), in the neurology consultation of the Institute of Neurology and Neurosurgery, and had a diagnosis of neurological diseases. Results: Of the total number of patients, 45.1 percent manifested to medicate themselves; it was more frequent in women (63.1 percent), with a predominance in non-university patients (90 percent) and housewives (54.3 percent). The main symptom that motivated self-medication was headache (30.4 percent), while nonopioid analgesics made up the group with the highest consumption (23.9 percent). Latency regarding the diagnosis of neurological diseases in the self-medicated group was between ten and nineteen months higher than in the non-self-medicated group. Conclusions: Self-medication is a very frequent practice, which caused the diagnostic and therapeutic delay of various neurological diseases of low incidence, ruining their adequate evolution(AU)

Ozono como método de desinfección del ambiente hospitalario / Ozone a method of disinfection of the environment of hospitals

Resumen Justificación y objetivo: Las infecciones asociadas a la asistencia sanitaria elevan los costos, prolongan la estadía hospitalaria e incrementan la mortalidad, por lo que se hace necesaria la desinfección ambiental como método de disminuir este problema. El objetivo del estudio fue evaluar el impacto del uso del ozono para la desinfección ambiental, en áreas de alto riesgo hospitalario. Métodos: Se realizó una intervención para la desinfección de ambiente con el empleo de equipos de ozono en áreas de alto riesgo del Instituto de Neurología y Neurocirugía de Cuba, y se evaluó el resultado mediante las siguientes variables: estado microbiológico ambiental, número de fallecidos en general, por sepsis, y consumo de antibióticos durante enero - junio de 2019. Resultados: De las 12 áreas evaluadas, previo a la aplicación de ozono ambiente, teniendo en cuenta la cantidad de unidades formadoras de colonias por m3, 2 se clasificaron como ambiente no aceptable, 3 como ambiente aceptable, 7 como ambiente limpio. Posterior a la aplicación de ozono, se obtuvo en las 12, resultados correspondientes a ambiente muy limpio. El consumo de antibióticos disminuyó a un tercio en las áreas expuestas evaluadas y se elevó en las no expuestas. Hubo menor número de fallecidos que en similar periodo del año anterior a la intervención. Conclusiones: La desinfección ambiental con ozono en áreas hospitalarias de alto riesgo fue útil para generar ambientes limpios, y favorecer la disminución del consumo de antibióticos, la estadía hospitalaria y la mortalidad por infecciones asociadas a la asistencia sanitaria.

Summary Background and aim: Health care-associated infections raise costs, prolong hospital stays and increase mortality, making environmental disinfection necessary as a method of reducing this problem. The aim of this study was to evaluate the impact of the use of ozone for environmental disinfection in areas of high hospital risk. Methods: An intervention was carried out to disinfect the environment with the use of ozone equipment in high-risk areas of the Cuban Institute of Neurology an Neurosurgery and the result was evaluated using the following variables: environmental microbiological status, number of deaths in general and for sepsis, and consumption of antibiotics from January to June 2019. Results: Of the 12 areas evaluated, prior to the application of ozone, taking into account the number of colony forming units per m3, 2 were classified as unacceptable, 3 as an acceptable environment and 7 as a clean environment . After the ozone application, the results of the 12 areas showed a very clean environment. Antibiotic use decreased to a third in the exposed areas evaluated and rose in the unexposed areas. There were fewer deaths than in a similar period of the year prior to the intervention. Conclusions: Environmental disinfection with ozone in high-risk hospital areas is useful to generate clean environments, and favor the decrease in antibiotic consumption and mortality due to infections associated with healthcare.

Epilepsia farmacorresistente. Experiencia quirúrgica en el Instituto de Neurología y Neurocirugía (2012-2018) / Drug-resistant epilepsy. Experience with surgical treatment at the Institute of Neurology and Neurosurgery, Havana, Cuba (2012-2018)

RESUMEN Introducción: La epilepsia es la alteración neurológica más frecuente en la población general. El objetivo de la cirugía de epilepsia es garantizar la ausencia o disminución de crisis, lo que se logra en el 80 % de los pacientes. Objetivo: Evaluar la evolución posquirúrgica y factores de buen pronóstico de los pacientes intervenidos de epilepsia farmacorresistente (EFR) en el Instituto de Neurología y Neurocirugía. Material y Métodos: Se realizó un estudio observacional descriptivo retro y prospectivo en el Instituto de Neurología y Neurocirugía entre enero de 2012 a mayo de 2018. Resultados: La edad del 44,8 % de los pacientes estuvo entre 21 y 30 años, el 62,1 % era del sexo masculino y el 82,8 % tenía color de piel blanca, el 31 % presentó entre 11 y 20 años de evolución. En el 75,9 % la epilepsia era lesional, el 55,2 % con localización temporal y en el 86,2 % se demostró congruencia clínica. Se utilizaron técnicas resectivas en 87,6 %. El 82,8 % no presentó crisis postoperatorias. En 62,1 % no se presentaron complicaciones. A los 3, 6 y 12 meses después de la intervención entre el 55 al 82 % de los pacientes estudiados se clasificaron como Engel clase I y clase II. Conclusiones: En nuestro estudio predominó la utilización de técnicas resectivas y la ausencia de crisis posterior a la cirugía. No se registró relación significativa entre la evolución posquirúrgica, la etiología de la epilepsia y la congruencia clínica. La presencia de lesión focal en el lóbulo temporal constituyó un factor de buen pronóstico.

ABSTRACT Introduction: Epilepsy is the most frequent neurological alteration in the general population. The objective of epilepsy surgery is to guarantee the absence or the decrease of seizures which is achieved in 67 % and 80 % of patients. Objective: To evaluate the surgical outcome and the factors for good outcome in patients with drug-resistant epilepsy (DRE) who underwent surgical treatment at the Institute of Neurology and Neurosurgery. Material and Methods: A retrospective prospective observational study was conducted at the Institute of Neurology and Neurosurgery between January 2012 and May 2018. Results: Of all the patients studied, 44,8 % were between 21 and 30 years old, 62,1 % were male and 82,8 % were white. Also, 31 % were between 11 and 20 years of follow-up. Epilepsy was lesional in 75,9 % of patients whereas in 55,2 % of them it was located in the temporal lobe; clinical congruence was demonstrated in 86,2 % of patients. Resective techniques were used in 87,6 % of them. Besides, 82,8 % had no postoperative seizures. There were no complications in 62,1 % of patients. On the other hand, 55 and 82 % of the patients studied were classified as Engel Class I and Engel Class II at 3, 6 and 12 months after surgical intervention. Conclusions: The use of resective techniques and the absence of seizures after surgery predominated in our study. No significant relationship was found between surgical outcome, etiology of epilepsy and clinical congruence. The presence of a focal lesion of the temporal lobe was a factor for good outcome.

Comparison of the clinical and genetic features of amyotrophic lateral sclerosis across Cuban, Uruguayan and Irish clinic-based populations.

OBJECTIVES: This study compares the clinical characteristics of patients with amyotrophic lateral sclerosis (ALS) within three clinic-based populations from Cuba, Uruguay and Ireland and determines the impact of known ALS-associated genetic variants on phenotypic manifestations within the Cuban population. METHODS: Demographic and clinical information was collected on 115 Cuban, 220 Uruguayan and 1038 Irish patients with ALS attending national specialist clinics through 1996-2017. All Cuban patients and 676 Irish patients underwent next-generation DNA sequencing and were screened for the pathogenic C9orf72 repeat expansion. RESULTS: The mean age of onset was younger in the Cuban (53.0 years, 95% CI 50.4 to 55.6) and Uruguayan (58.2 years, 95% CI 56.5 to 60.0) populations compared with the Irish population (61.6 years, 95% CI 60.9 to 62.4). No differences in survival between populations were observed. 1.7 % (95% CI 0.6 to 4.1) of Cubans with ALS carried the C9orf72 repeat expansion compared with 9.9% (95% CI 7.8 to 12.0) of Irish patients with ALS (p=0.004). Other known variants identified in the Cuban population included ANG (one patient), CHCHD10 (one patient) and DCTN1 (three patients). CONCLUSIONS AND RELEVANCE: This study is the first to describe the clinical characteristics of ALS in Cuban and Uruguayan populations and report differences between the Cuban and Irish genetic signature in terms of known ALS-associated genetic variants. These novel clinical and genetic data add to our understanding of ALS across different and understudied populations.

Association of status redox with demographic, clinical and imaging parameters in patients with Huntington's disease.

UNLABELLED: Huntington's disease (HD) is an autosomal dominant, progressive neurodegenerative disorder, caused by an expanded trinucleotide CAG sequence of the huntingtin (Htt) gene, which encodes a stretch of glutamines in the Htt protein. The mechanisms of neurodegeneration associated with the accumulation of Htt aggregates still remains unclear. OBJECTIVES: To determine oxidative stress biomarkers in HD patients and their relationship with clinical, demographic and neuroimaging parameters. DESIGN AND METHODS: Fourteen patients and 39 controls paired by age and sex participated in this study. Oxidative damage was assayed in blood by measuring malondialdehyde (MDA) and advanced oxidative protein products (AOPPs). Antioxidant status was determined by activities of superoxide dismutase (SOD), catalase (CAT), glutathione peroxidase (GPx), glutathione reductase (GR), reduced glutathione (GSH), protein thiols and total antioxidant capacity (FRAP). The Unified Huntington Disease Rating Scale (UHDRS) and neuroimaging studies were also employed. RESULTS: MDA, AOPP and GPx were significantly increased in HD patients with respect to the control group, while GR activity was decreased. FRAP correlated with age of disease onset, AOPP with motor severity (UHDRS score), age of patients and age of disease onset. Caudate atrophy was associated with lower plasma concentrations of GSH. CONCLUSIONS: These findings point to a redox imbalance in HD patients. GR activity could be a potential biomarker for symptom onset in asymptomatic gene carriers, while plasmatic GSH could be useful in monitoring the progression of neurodegeneration - as an expression of caudate atrophy - during the course of the disease.

Propuesta de programa de la residencia de neurología en Cuba / A proposal of residency neurology program in Cuba

Objetivo: Proponer modificaciones para estandarizar y actualizar el programa de la especialidad de neurología en Cuba.Desarrollo: Los cambios mundiales en la neurología han traído consigo la necesidad de un análisis profundo, integral y actualizado de laorganización y los contenidos del programa de la especialidad de neurología en Cuba. En las últimas dos décadas varias instituciones docentes han hecho ajustes imprescindibles, pero no uniformes, al programa al programa vigente desde el año 1979 para garantizar la adecuada formación del especialista en neurología. Este documento pretende servir de referencia con respecto a: 1) Denominación oficial de la especialidad y requisitos de la titulación; 2) Definición de la especialidad y rasgos de su evolución histórica; 3) Objetivos generales y competencias de la formación; 4) Estrategia docente, 5) Distribución y duración de los períodos de formación; 6) Contenidos específicosde formación (incluyendo los objetivos); 7) Bibliografía recomendada; 8) Lugares y recursos de formación; y 9) Sistema de evaluación. Se han revisado varios programas de residencia de otros países, los documentos nacionales al respecto, y varios artículos sobre el tema.Conclusiones: El análisis crítico por los profesores y residentes en relación a los objetivos, estrategias y contenidos incluidos en este artículo contribuirá a que el programa de residencia de neurología cumpla los estándares más exigentes y la misión de lograr la excelencia en el cuidado del paciente, la educación y la investigación(AU)

Objective: To review and formulate recommendations of cuban neurology residency curriculum for standardization and update.Development: World neurology practice changes cause a profound, integral and update analysis about organization and contents of neurology specialty program in Cuba. In last two decades some docents national units make necessary, but not uniform, adjustments to1979 year approved program with the purpose to achieve adequate formation of neurologists. This document pretends to serve as reference regarding: 1) Official specialty denomination and requisites of qualifications; 2) Specialty definition and some historiccharacteristic; 3) General objective and formation competences; 4) Docent strategy; 5) Distribution and duration of formation periods; 6)Specific formation contents (including objectives); 7) Recommended bibliography; 8) Formation places and resources; and 9) Evaluation system. A review was carried out about some residency programs of other countries, national documents, and various articles about this issue.Conclusions: Understanding and critical analysis for professors and residents about objectives, strategies, and contents included in this article may contribute to fulfill most exigent standards and mission of excellence for patients care, education and investigation(AU)

Efecto del zumo de Morinda citrifolia L (noni) en modelos de analgesia / Effect of Morinda citrifolia L. (noni) in analgesic models

Introducción: Morinda citrifolia L (noni) ha despertado gran interés y expectativa dentro de la población cubana debido a las propiedades medicinales que se le atribuyen. Investigaciones realizadas evidencian las propiedades analgésicas de algunas de sus partes. Objetivos: evaluar el efecto del zumo de noni en diferentes modelos de analgesia. Métodos: se utilizaron dosis (450, 900 y 1 800 mg/kg) del zumo de noni, a partir de contenido en peso seco; se administró por vía intraperitoneal a ratones OF1 en el modelo de irritación peritoneal por ácido acético 0,6 por ciento y se cuantificó el número de contorsiones o estiramientos. Además, se utilizó el modelo del plato caliente y el de la retirada de la cola. Resultados: el zumo de noni fue efectivo de manera dependiente de la dosis en reducir el número de contorsiones inducidas por el ácido acético. En los modelos del plato caliente y de retirada de la cola, solo la dosis más alta prolongó de manera estadísticamente significativa el tiempo de reacción. Conclusiones: los resultados sugieren que el efecto analgésico de noni es fundamentalmente de mecanismo periférico

Introduction: Morinda citrifolia L (noni) has aroused great interest and expectations in the Cuban population due to attributed medicinal properties. Several research works have suggested the analgesic effect of several parts of the plant. Objectives: to evaluate the effect of Noni juice in different analgesic models. Methods: there were used 450, 900, and 1 800 mg/kg doses of the juice, based on the dry content weight. They were administered intraperitonealy to adult male mice OF1 in the peritoneal irritation model induced by acetic acid at 0,6 percent concentration, and the number of contorsions or stretchings was quantified. Additionally, the hot plate and the tail immersed in hot water models were applied. Results: the noni juice was effective in reducing the number of contortions induced by the acetic acid in a dose-dependent manner. Just the highest dose of the juice increased significantly the time of reaction in the hot plate and in the tail immersion test. Conclusions: these results suggest that the analgesic effect of Noni juice is basically peripheral

Efecto del zumo de Morinda citrifolia L (noni) en modelos de analgesia / Effect of Morinda citrifolia L. (noni) in analgesic models

Introducción: Morinda citrifolia L (noni) ha despertado gran interés y expectativa dentro de la población cubana debido a las propiedades medicinales que se le atribuyen. Investigaciones realizadas evidencian las propiedades analgésicas de algunas de sus partes. Objetivos: evaluar el efecto del zumo de noni en diferentes modelos de analgesia. Métodos: se utilizaron dosis (450, 900 y 1 800 mg/kg) del zumo de noni, a partir de contenido en peso seco; se administró por vía intraperitoneal a ratones OF1 en el modelo de irritación peritoneal por ácido acético 0,6 por ciento y se cuantificó el número de contorsiones o estiramientos. Además, se utilizó el modelo del plato caliente y el de la retirada de la cola. Resultados: el zumo de noni fue efectivo de manera dependiente de la dosis en reducir el número de contorsiones inducidas por el ácido acético. En los modelos del plato caliente y de retirada de la cola, solo la dosis más alta prolongó de manera estadísticamente significativa el tiempo de reacción. Conclusiones: los resultados sugieren que el efecto analgésico de noni es fundamentalmente de mecanismo periférico(AU)

Introduction: Morinda citrifolia L (noni) has aroused great interest and expectations in the Cuban population due to attributed medicinal properties. Several research works have suggested the analgesic effect of several parts of the plant. Objectives: to evaluate the effect of Noni juice in different analgesic models. Methods: there were used 450, 900, and 1 800 mg/kg doses of the juice, based on the dry content weight. They were administered intraperitonealy to adult male mice OF1 in the peritoneal irritation model induced by acetic acid at 0,6 percent concentration, and the number of contorsions or stretchings was quantified. Additionally, the hot plate and the tail immersed in hot water models were applied. Results: the noni juice was effective in reducing the number of contortions induced by the acetic acid in a dose-dependent manner. Just the highest dose of the juice increased significantly the time of reaction in the hot plate and in the tail immersion test. Conclusions: these results suggest that the analgesic effect of Noni juice is basically peripheral(AU)

Presentación de un caso clínico de atrofia multisistémica y actualización de criterios diagnósticos / Multisystem atrophy and diagnostic criteria updating: A clinical case presentation

La atrofia multisistémica constituye un trastorno neurodegenerativo esporádico de etiología no precisada que se caracteriza por parkinsonismo, trastornos cerebelosos, disfunción autonómica y piramidalismo; los hallazgos patológicos comprenden pérdida celular y gliosis en las neuronas estriatonígricas, olivopontocerebelosas y autonómicas; y la presencia de inclusiones intracitoplasmáticas oligodendrogliales y neuronales, ubiquitina, tau y alfasinucleína positivas. Afecta tanto a hombres como a mujeres, con inicio en la sexta década de la vida como promedio y una prevalencia de 4/100 000. Se presentaron los últimos criterios diagnósticos de atrofia multisistémica y el caso clínico de un paciente de 65 años con un cuadro progresivo, de 4 años de evolución, ataxia cerebelosa progresiva, síndrome rígido acinético, disfunción autonómica, signos piramidales y mala respuesta a la levodopa, con imágenes de resonancia magnética que muestran atrofia de vermis, hemisferios cerebelosos, tallo cerebral (puente) e hipointensidad de ambas regiones putaminales en t2. Se concluyó el caso con el diagnóstico de atrofia multisistémica tipo C

The multisystem atrophy is a sporadic neurodegenerative disorder of unknown origin characterized by parkinsonism, cerebellar disorders, autonomic dysfunction and pyramidal disease, provoked by a cellular loss and gliosis in the nigrostriatal, olivopontocerebellar and autonomic neurons and the presence of oligodendroglia and neuronal intracytoplasmic positive inclusions, ubiquitin, tau and alpha-sinuclein affecting men and women starting as average during the sixth decade of life and a prevalence of 4/100 000. The last diagnostic criteria of multisystem atrophy were showed as well as the clinical case of a patient aged 65 with a progressive picture of 4 years of evolution, progressive cerebellar ataxia, a rigid akinetic syndrome autonomic dysfunction, pyramidal signs and a poor response to levodopa with magnetic resonance images showing vermis atrophy, cerebellar hemispheres, cerebral stem (bridge) and hipointensity of both putamen regions in T2. We conclude that case was diagnosed with type C multisystem atrophy

Presentación de un caso clínico de atrofia multisistémica y actualización de criterios diagnósticos / Multisystem atrophy and diagnostic criteria updating: A clinical case presentation

La atrofia multisistémica constituye un trastorno neurodegenerativo esporádico de etiología no precisada que se caracteriza por parkinsonismo, trastornos cerebelosos, disfunción autonómica y piramidalismo; los hallazgos patológicos comprenden pérdida celular y gliosis en las neuronas estriatonígricas, olivopontocerebelosas y autonómicas; y la presencia de inclusiones intracitoplasmáticas oligodendrogliales y neuronales, ubiquitina, tau y alfasinucleína positivas. Afecta tanto a hombres como a mujeres, con inicio en la sexta década de la vida como promedio y una prevalencia de 4/100 000. Se presentaron los últimos criterios diagnósticos de atrofia multisistémica y el caso clínico de un paciente de 65 años con un cuadro progresivo, de 4 años de evolución, ataxia cerebelosa progresiva, síndrome rígido acinético, disfunción autonómica, signos piramidales y mala respuesta a la levodopa, con imégenes de resonancia magnética que muestran atrofia de vermis, hemisferios cerebelosos, tallo cerebral (puente) e hipointensidad de ambas regiones putaminales en t2. Se concluyó el caso con el diagnóstico de atrofia multisistémica tipo C(AU)

The multisystem atrophy is a sporadic neurodegenerative disorder of unknown origin characterized by parkinsonism, cerebellar disorders, autonomic dysfunction and pyramidal disease, provoked by a cellular loss and gliosis in the nigrostriatal, olivopontocerebellar and autonomic neurons and the presence of oligodendroglia and neuronal intracytoplasmic positive inclusions, ubiquitin, tau and alpha-sinuclein affecting men and women starting as average during the sixth decade of life and a prevalence of 4/100 000. The last diagnostic criteria of multisystem atrophy were showed as well as the clinical case of a patient aged 65 with a progressive picture of 4 years of evolution, progressive cerebellar ataxia, a rigid akinetic syndrome autonomic dysfunction, pyramidal signs and a poor response to levodopa with magnetic resonance images showing vermis atrophy, cerebellar hemispheres, cerebral stem (bridge) and hipointensity of both putamen regions in T2. We conclude that case was diagnosed with type C multisystem atrophy(AU)

Guías de práctica clínica para las enfermedades cerebrovasculares / Clinic practical guides for cerebrovascular diseases

Se presentan las guías de práctica clínica para las enfermedades cerebrovasculares. Incluye aspectos como su concepto y clasificación, datos epidemiológicos en el mundo y en Cuba, así como su diagnóstico, clasificación, complicaciones y tratamiento. Se ofrece la forma y frecuencia de la evaluación de su aplicación e incluye instrumentos para medir calidad de vida de los enfermos con accidentes cerebrovasculares(AU)

The clinic practical guides for cerebrovascular diseases are presented. They include different aspects as its concept, classification, and epidemiological data in Cuba as well as worldwide. They also offer its diagnosis, classification, complications and treatment. The frequency of assessment of its application including the tools to measure the quality of life in patients with cerebrovascular accident and the way to proceed with them are shown(AU)