Impacto de la superación profesional en la meteoro labilidad de la enfermedad cerebrovascular / Climate change and cardiovascular disease: impact of professional development

Introducción: La enfermedad cerebrovascular (ECV) es una urgencia neurológica que en ocasiones se encuentra influenciada por los cambios del tiempo. La ECV isquémica es una emergencia médica con una ventana estrecha para su diagnóstico y tratamiento. El daño generado por esta enfermedad se estima mediante de la mortalidad, discapacidad e impacto social. Objetivo: Proponer un programa de capacitación basado en los efectos fisiopatológicos que provocan en los pacientes, cambios de tiempo y ritmo circadiano en el contexto de la adaptación al cambio climático. Método: Se desarrolló un estudio multietápico prospectivo en el Hospital Universitario Mártires del 9 de Abril del municipio Sagua la Grande entre los años 1993 a 2017. En la primera etapa se seleccionó un universo de 52 profesionales de la salud que atendieron a estos pacientes; en la segunda, 48 y en la tercera, 61. Resultados: El nivel de conocimiento del personal de salud en una primera etapa era inadecuado (78,85 %); en la tercera, este porcentaje disminuyó (14,75 %). Como resultado de la capacitación mejoraron los tiempos de atención a los pacientes con ECV a su llegada al hospital, y en la tercera, más del 50 % de los enfermos fueron atendidos antes de las seis horas de inicio de los síntomas. Conclusiones: La capacitación sobre ECV basada en los efectos fisiopatológicos que provocan en los pacientes el cambio climático y el ritmo circadiano disminuyó el tiempo de espera, y se asoció con una reducción de la morbilidad y la mortalidad, después de aplicar este programa.

Introduction: cerebrovascular disease (CVD) is a neurological emergency that is sometimes influenced by climate changes. Ischemic CVD is a medical emergency with a narrow window for diagnosis and treatment. The damage generated by this disease is estimated through mortality, disability and social impact. Objective: to propose a training program based on the pathophysiological effects in patients caused by changes in time and circadian rhythm in the context of adaptation to climate change. Methods: a multistage prospective study was conducted at "Mártires del 9 de Abril" University Hospital in Sagua la Grande municipality between 1993 and 2017. A universe of health professionals who cared for these patients was selected as follows: 52 in the first stage; 48 in the second stage, and 61 in the third ones. Results: the level of knowledge of health personnel in the first stage was inadequate (78.85%); this percentage decreased in the third stage (14.75%). Care times for CVD patients improved upon arrival at the hospital as a result of the training, as well as in the third ones, more than 50% of the patients were seen within six hours of the onset of symptoms. Conclusions: CVD training based on the pathophysiological effects of climate change and circadian rhythm on patients decreased waiting time, and was associated with reduced morbidity and mortality, after applying this program.

Leigh syndrome in an infant: autopsy and histopathology findings

Leigh syndrome is an inherited neurodegenerative disorder of infancy that typically manifests between 3 and 12 months of age. The common neurological manifestations are developmental delay or regression, progressive cognitive decline, dystonia, ataxia, brainstem dysfunction, epileptic seizures, and respiratory dysfunction. Although the disorder is clinically and genetically heterogeneous, the histopathological and radiological features characteristically show focal and bilaterally symmetrical, necrotic lesions in the basal ganglia and brainstem. The syndrome has a characteristic histopathological signature that helps in clinching the diagnosis. We discuss these unique findings on autopsy and radiology in a young infant who succumbed to a subacute, progressive neurological illness suggestive of Leigh syndrome. Our case highlights that Leigh syndrome should be considered in the differential diagnosis of infantile-onset, subacute neuroregression with dystonia and seizures, a high anion gap metabolic acidosis, normal ketones, elevated lactates in blood, brain, and urine, and bilateral basal ganglia involvement.

Moyamoya disease / Enfermedad de Moyamoya

Moyamoya disease is a chronic neurovascular disease characterized by progressive bilateral stenosis of the internal carotid arteries with abnormal formation of new abnormal blood vessels whose blockage of blood flow causes a variety of clinical manifestations and complications associated with acute cerebrovascular events (ischemic or hemorrhagic), which may even be fatal. This article seeks to describe this entity, its incidence, prevalence, forms of presentation, therapeutic measures and prognosis through a clinical case report. (Acta Med Colomb 2020; 45. DOI:http://doi.org/10.36104/amc.2020.1424).

La enfermedad de Moyamoya es una enfermedad neurovascular crónica caracterizada por una estenosis progresiva bilateral de las arterias carótidas internas, con la formación anormal de nuevos vasos sanguíneos, cuyo bloqueo en flujo sanguíneo ocasiona variadas manifestaciones clínicas y complicaciones asociadas a eventos cerebrovasculares (isquémicos o hemorrágicos) agudos que pueden llegar a ser incluso mortales. El presente artículo pretende realizar una aproximación acerca de esta entidad, su incidencia, prevalencia, formas de presentación y medidas terapéuticas y pronostico mediante el reporte de un caso clínico.(Acta Med Colomb 2020; 45. DOI:https://doi.org/10.36104/amc.2020.1424).

El sistema antioxidante del glutatión en la etiopatología de la disfunción nigro-estriatal / Glutathione antioxidant system in the etiopathology of nigrostriatal dysfunction

La enfermedad de Parkinson es una enfermedad neurodegenerativa crónica que afecta a las personas de la tercera edad. En una minoría de los casos la enfermedad es de origen genético pero en el resto, la causa es idiopática. En este sentido, la acumulación de los radicales libres y la pérdida de la homeostasis del glutatión se han señalado como posibles agentes causales. El presente texto se propuso revisar las evidencias experimentales que apoyan la participación de los radicales libres y la pérdida de la homeostasis del glutatión en el comienzo y la progresión de la degeneración de la substantianigrapars compacta. El estrés oxidativo en la enfermedad de Parkinson´s puede estar relacionado con las propiedades pro-oxidantes intrínsecas de la dopamina y elevadas concentraciones de hierro en la substantianigrapars compacta, que promueven la oxidación de la dopamina y la generación de especies reactivas del oxígeno. Cualquier evento que desencadene estos mecanismos, genera un daño celular. La disminución del glutatión es una de las alteraciones bioquímicas más tempranas, detectadas en asociación con la enfermedad de Parkinson y se ha relacionado con la inhibición del complejo I de la cadena de transporte mitocondrial, daño oxidativo, activación glial, entre otros que favorecen la neurodegeneración. Estas evidencias sugieren la necesidad de mantener la homeostasis del glutatión en el sistema dopaminérgico y su vínculo con la etiología de la degeneración nigro-estriatal, lo que tiene una potencial aplicación en la práctica clínica.

Parkinson's disease is a chronic neurodegenerative condition affecting elderly persons. In a minority of cases the disease has a genetic origin, but in most the cause is idiopathic. Accumulation of free radicals and loss of glutathione homeostasis have been pointed at as possible causal agents. The purpose of the study was to review experimental evidence supporting the involvement of free radicals and loss of glutathione homeostasis in the outset and progress of substantia nigra pars compacta degeneration. Oxidative stress in Parkinson's disease may be related to the intrinsic pro-oxidant properties of dopamine and high iron concentrations in the substantia nigra pars compacta, promoting dopamine oxidation and the generation of reactive oxygen species. Any event triggering these mechanisms will cause cell damage. Glutathione reduction is one of the earliest biochemical alterations detected in association with Parkinson's disease, and it has been related to the inhibition of complex I of the mitochondrial transport chain, oxidative damage and glial activation, among other factors leading to neurodegeneration. This evidence points to the need to maintain glutathione homeostasis in the dopaminergic system, as well as its relationship to the etiology of nigrostriatal degeneration, of potential application in clinical practice.

Comparison on different strategies for treatments of hypertensive hemorrhage in the basal ganglia region with a volume of 25 to 35ml / Comparação de diferentes estratégias no tratamento da hemorragia hipertensiva da região dos gânglios da base com volume de 25 a 35ml

PURPOSE: To compare curative effect of different treatments for hypertensive cerebral hemorrhage of 25 to 35ml. METHODS: In this study, 595 cases were enrolled and grouped regarding treatments including conservative treatment, evacuation with microinvasive craniopuncture technique within 6h and 6-48h after the attack. RESULTS: After follow up for three months after the attack, the assessment based on the Activity of Daily Living (ADL) indicated no significant difference among conservative treatment and surgical interventions (p>0.05). However, surgical interventions showed advantages of shorter hospitalization, quick removal of hematoma and obvious reduction of cost. CONCLUSION: The microinvasive craniopuncture technique to drain the hematoma within 6-48h may be a good way in treating hypertensive hemorrhage of basal ganglia region.

OBJETIVO: Comparar o efeito curativo de diferentes tratamentos da hemorragia hipertensiva cerebral de 25 a 35ml. MÉTODOS: Foram analisados 595 casos agrupados segundo tratamento conservador e evacuação com técnica de punção transcraniana dentro de 6h ou de 6 às 48h do início do quadro clínico. RESULTADOS: O seguimento após três meses e avaliado pelo Escore de Atividade de Vida Diário, indicou que não houve diferenças significantes entre os tratamentos conservador e cirúrgico (p>0.05) O tratamento cirúrgico mostrou vantagem com hospitalização mais curta e redução de custos. CONCLUSÃO: A técnica de punção transcraniana para drenagem de hematoma dos núcleos da base pode ser uma boa alternativa de tratamento.

Manganese and Parkinson's disease: a critical review and new findings / Manganês e doença de Parkinson: uma revisão crítica e novas descobertas

The goal of this review was to examine whether chronic Mn exposure produces dopamine neuron degeneration and PD or whether it has a distinct neuropathology and clinical presentation. I reviewed available clinical, neuroimaging, and neuropathological studies in humans and nonhuman primates exposed to Mn or other human conditions that result in elevated brain Mn concentrations. Human and nonhuman primate literature was examined to compare clinical, neuroimaging, and neuropathological changes associated with Mn-induced parkinsonism. Clinical, neuroimaging, and neuropathological evidence was used to examine whether Mn-induced parkinsonism involves degeneration of the nigrostriatal dopaminergic system as is the case in PD. The overwhelming evidence shows that Mn-induced parkinsonism does not involve degeneration of midbrain dopamine neurons and that l-dopa is not an effective therapy. New evidence is presented on a putative mechanism by which Mn may produce movement abnormalities. Confirmation of this hypothesis in humans is essential to make rational decisions about treatment, devise effective therapeutic strategies, and set regulatory guidelines.

O objetivo desta revisão foi examinar se a exposição crônica ao Mn produz degeneração do neurônio pela dopamina e DP ou se é apenas uma apresentação neuropatológica e clínica diferente. Foram revisados estudos clínicos, de neuroimagens e neuropatológicos disponíveis sobre humanos e primatas expostos ao Mn ou outras condições humanas que resultam em concentrações elevadas de Mn no cérebro. Foi examinada a literatura sobre humanos e primatas e comparadas as mudanças clínicas de neuroimagem e neuropatológicas associadas com o "parkinsonimo" induzido por Mn, envolvendo a degeneração do sistema dopaminérgico nigro-estriatal como no caso da DP. as evidências decisivas mostram que o "parkinsonismo" induzido pelo Mn não envolve a degeneração dos neurônios de dopamina do mesencéfalo e que o dopa-1 não é uma terapia eficaz. Novas evidências estão presentes em um mecanismo putativo pelo qual o Mn pode produzir anormalidades de movimento. A confirmação desta hipótese em humanos é essencial para tomar decisões adequadas sobre o tratamento, planejar estratégias terapêuticas eficazes e estabelecer guias regulatórios.