La atrofia cerebral como hallazgo o factor predictor del deterioro cognitivo en el envejecimiento normal / Cerebral Atrophy as a Finding of Predictive Factor for Cognitive Impairment in Normal Aging

Introducción: En el curso del envejecimiento es conocida la existencia de un patrón complejo de cambios estructurales cerebrales, conductuales y cognitivos, en ocasiones relacionados con enfermedades neurológicas y psiquiátricas. Objetivo: Determinar la posible relación de causalidad de la atrofia cerebral en la aparición del deterioro cognitivo en el curso del envejecimiento normal. Métodos: Se desarrolló un estudio retrospectivo, transversal, descriptivo y observacional. El universo estuvo conformado por el total de los pacientes de ambos sexos con edades comprendidas entre 35-74 años de edad, con indicaciones previas de tomografía computarizada de cráneo y cuyos resultados fueron informados con signos de atrofia cerebral, cuya cifra ascendió a 733. Resultados: El grupo de edad que predomino fue el de 45-54 años (35,3 por ciento), así como las pacientes del sexo femenino (66,3 por ciento). El 27,7 por ciento tenía como nivel de escolaridad el técnico medio superior y 36,2 por ciento fueron pacientes amas de casa. El 99,7 por ciento fueron diestros. Un total de 368 voluntarios presentaron deterioro cognitivo y 365 sujetos no evidenciaron declive en las funciones exploradas. Las funciones de atención y cálculo y retención verbal a corto plazo fueron las que se vieron más afectadas, seguidas de orientación espacial y memoria verbal de fijación. Conclusiones: No se logró establecer una relación de causalidad significativa entre el diagnóstico radiológico de atrofia cerebral y la presencia de deterioro cognitivo(AU)

Introduction: In the course of aging, the existence of a complex pattern of behavioral, cognitive and cerebral structural changes is known, sometimes related to neurological and psychiatric diseases. Objective: To determine the possible causal relationship of cerebral atrophy with the onset of cognitive impairment in the course of normal aging. Methods: A retrospective, cross-sectional, descriptive and observational study was carried out. The study universe consisted of all patients of both sexes aged 35-74 years, with previous indications for cranial computed tomography and whose results were reported with signs of cerebral atrophy, which numbered 733. Results: The predominant age group was 45-54 years old (35.3percent), as well as female patients (66.3percent). The educational level of 27.7percent of the patients was technical high school and 36.2percent were housewife patients. A total of 99.7percent were right-handed. A total of 368 volunteers showed cognitive impairment and 365 subjects showed no decline in the tested functions. The functions of attention and calculation, as well as short-term verbal retention, were the most affected, followed by spatial orientation and speech retention memory. Conclusions: No significant causal relationship was established between the radiological diagnosis of cerebral atrophy and the presence of cognitive impairment(AU)

Triphasic waves and brain atrophy in patients with acute encephalopathy / Ondas trifásicas y atrofia cerebral en pacientes con encefalopatía aguda

Abstract Introduction: Triphasic waves (TW) constitute an electroencephalographic pattern associated with certain kinds of encephalopathy. Brain atrophy may be a predisposing factor linked with TW. Objective: To compare the degree of brain atrophy and white matter disease between patients with acute encephalopathy with and without TW. Methods: A retrospective observational study including adult patients with encephalopathy, with and without TW, hospitalized between 2016 and 2017. The degree of brain atrophy and white matter lesion were defined using the Global Cortical Atrophy and Age Related White Matter Changes (ARWMC) scales, respectively. Scores were compared between groups. Mortality rates were registered. Results: Sixteen patients with TW were identified matched by age and sex with 30 patients without TW. The mean age was 80 years in the TW group. Women represented 87.5%. Multifactorial encephalopathy was the most frequent diagnosis followed by metabolic encephalopathy. Patients with TW had more brain atrophy (10.43 vs 6.9, p= 0.03). Mean ARWMC was 9.43±6.5 and 8.5 ±7.89 in patients with and without TW respectively (p= 0.5). Mortality rate was higher in the TW group (31.25 vs 6.66% p= 0.02). Conclusions: Patients with acute encephalopathy and TW had higher degree of cerebral atrophy. It is possible that this structural alteration predisposes to the appearance of TW. There was no significant difference in white matter lesion degree. The mortality of the TW group was high, so future studies are necessary to determine their prognostic value.

Resumen Introducción: Las ondas trifásicas (OT) constituyen un patrón electroencefalográfico asociado con diversas encefalopatías. La atrofia cerebral podría predisponer a su aparición. Objetivo: Comparar el grado de atrofia cerebral y de lesión de sustancia blanca en pacientes con encefalopatía aguda con y sin OT. Métodos: Estudio observacional retrospectivo, incluyó pacientes adultos con encefalopatía aguda con y sin OT internados entre 2016 y 2019. El grado de atrofia cerebral y de lesión de sustancia blanca se definieron según las escalas Global Cortical Atrophy y Age Related White Matter Changes (ARWMC), respectivamente. Se compararon los puntajes entre grupos. Se registró la mortalidad. Resultados: Se identificaron 16 pacientes con OT y 30 sin OT pareados según edad y sexo. La edad promedio del grupo con OT fue 80 años. El 87.5% fueron mujeres. La encefalopatía multifactorial fue el diagnóstico más frecuente seguido de la encefalopatía metabólica. El grado de atrofia fue mayor en pacientes con OT (10.43 vs 6.9, p= 0.03). El puntaje ARWMC fue 9.43 ±6.5 y 8.5 ±7.89 en pacientes con y sin OT respectivamente (p= 0.5). La mortalidad fue mayor en el grupo con OT (31.25 vs 6.66% p= 0.02). Conclusiones: Pacientes con encefalopatía aguda y OT tuvieron mayor grado de atrofia cerebral. Dicha alteración estructural podría relacionarse con la aparición de OT. No hubo diferencias significativas en el grado de lesión de sustancia blanca. La mortalidad del grupo con OT fue elevada. Son necesarios estudios para determinar su valor pronóstico.

Valor pronóstico del glutamato en el líquido cefalorraquídeo en esclerosis múltiple / Prognostic value of cerebrospinal fluid glutamate in multiple sclerosis

Resumen El objetivo del trabajo fue evaluar la asociación entre el nivel de glutamato en el líquido cefalorraquídeo (LCR) al inicio de la enfermedad y la progresión de la enfermedad durante el seguimiento en una cohorte de pacientes con esclerosis múltiple (EM). Se determinaron niveles de glutamato (Glu) en LCR al inicio de la enfermedad. Se realizó una resonancia basal y durante el seguimiento cada 12 meses con el objeto de determinar el porcentaje de cambio de volumen cerebral (PCVC), grosor cortical (GC) y volumen le sional cerebral en secuencia T2 (VLT2). Los predictores primarios de interés fueron los niveles basales de Glu en LCR, PCVC Y GC, así como la progresión clínica de la enfermedad [medida por Expanded Disability Status Scale (EDSS) y tasa anual de recaídas]. Un total de 26 pacientes fueron incluidos. La concentración media de Glu fue de 5.3 ± 0.4 μM/l. Se encontró una asociación significativa entre concentraciones basales elevadas de Glu y la progresión del EDSS (b = 1.06, IC 95% 0.47-1.66, p = 0.003), así como también el PCVC (b = -0.71, IC 95% -0.56-1.38, p = 0.002) y CG (b = -0.15, IC 95% -0.06-0.33, p = 0.01). No se encontró asociación entre los niveles de Glu y la tasa anual de recaídas como tampoco el VLT2 (b = 0.08, IC 95% -0.11-0.43, p = 0.11 y b = 195, IC -39-330, p = 0.22, respectivamente). Los niveles aumentados de Glu se asociaron con un mayor cambio en el PCVC y progresión del EDSS durante el seguimiento.

Abstract. The objective of this study was to evaluate the association between glutamate (Glu) levels in cerebrospinal fluid (CSF) at disease onset and disease progression during follow up in a cohort of multiple sclerosis (MS) patients. Glu level was measured at disease onset (first relapse). MRI was obtained at baseline and follow-up (every 12 months) to determine the percent of brain volume change (PBVC), cortical thickness (CT), and T2 lesion volume (T2LV). The primary predictors of interest were baseline CSF Glu levels, PBVC and CT, as well as clinical disease progression [measured by Expanded Disability Status Scale (EDSS) and annualized relapse rate] during follow-up. A total of 26 MS patients were included. Mean concentration of Glu in CSF at diagnosis was 5.3 ± 0.4 μM/l. A significant association was observed between higher baseline levels of Glu and an increase in EDSS during follow up (b = 1.06, 95%CI 0.47-1.66, p = 0.003) as well as PBVC (b = -0.71 95%CI -0.56-1.38, p = 0.002) and CT (b = -0.15, 95%CI -0.06-0.33, p = 0.01). We did not observe an association between baseline Glu levels and relapse rate or T2LV during follow-up (b = 0.08, 95%CI -0.11-0.43, p = 0.11 and b = 195, 95%CI -39-330, p = 0.22, respectively). Higher Glu concentrations at disease onset were associated with an increase in PBVC and EDSS progression during follow-up in MS patients.

Regional brain atrophy is related to social cognition impairment in multiple sclerosis / La atrofia cerebral regional se relaciona con el deterioro de la cognición social en sclerosis múltiple

ABSTRACT Background: Multiple sclerosis exhibits specific neuropathological phenomena driving to both global and regional brain atrophy. At the clinical level, the disease is related to functional decline in cognitive domains as the working memory, processing speed, and verbal fluency. However, the compromise of social-cognitive abilities has concentrated some interest in recent years despite the available evidence suggesting the risk of disorganization in social life. Recent studies have used the MiniSEA test to assess the compromise of social cognition and have found relevant relationships with memory and executive functions, as well as with the level of global and regional brain atrophy. Objective: The present article aimed to identify structural changes related to socio-cognitive performance in a sample of patients with relapsing-remitting multiple sclerosis. Methods: 68 relapsing-remitting multiple sclerosis Chilean patients and 50 healthy control subjects underwent MRI scans and neuropsychological evaluation including social-cognition tasks. Total brain, white matter, and gray matter volumes were estimated. Also, voxel-based morphometry was applied to evaluate regional structural changes. Results: Patients exhibited lower scores in all neuropsychological tests. Social cognition exhibited a significant decrease in this group mostly related to the declining social perception. Normalized brain volume and white matter volume were significantly decreased when compared to healthy subjects. The regional brain atrophy analysis showed that changes in the insular cortex and medial frontal cortices are significantly related to the variability of social-cognitive performance among patients. Conclusions: In the present study, social cognition was only correlated with the deterioration of verbal fluency, despite the fact that previous studies have reported its link with memory and executive functions. The identification of specific structural correlates supports the comprehension of this phenomenon as an independent source of cognitive disability in these patients.

RESUMEN Antecedentes: La esclerosis múltiple presenta fenómenos neuropatológicos específicos que conducen a la atrofia cerebral global y regional. A nivel clínico, la enfermedad está relacionada con el deterioro funcional de los dominios cognitivos como la memoria de trabajo, la velocidad de procesamiento y la fluidez verbal. Sin embargo, el compromiso de las habilidades socio-cognitivas ha concentrado cierto interés en los últimos años debido a la evidencia disponible que sugiere el riesgo de desorganización en la vida social. Estudios recientes han utilizado la prueba MiniSEA para evaluar el compromiso de la cognición social y han encontrado relaciones relevantes con la memoria y funciones ejecutiva, así como con el nivel de atrofia cerebral global y regional. Objetivo: El presente artículo tiene como objetivo identificar cambios estructurales relacionados con el rendimiento sociocognitivo en una muestra de pacientes con esclerosis múltiple recurrente-remitente. Métodos: 68 pacientes Chilenos con esclerosis múltiple recurrente-remitente y 50 sujetos de control sanos se sometieron a resonancias magnéticas y evaluación neuropsicológica, incluidas las tareas de cognición social. Se estimaron los volúmenes cerebrales totales, de materia blanca y materia gris. Además, se aplicó la morfometría basada en vóxel para evaluar los cambios estructurales regionales. Resultados: Los pacientes muestran puntuaciones más bajas en todas las pruebas neuropsicológicas. La cognición social exhibe una disminución significativa en este grupo principalmente relacionada con la disminución de la percepción social. El volumen normalizado del cerebro y el volumen de la materia blanca disminuyeron significativamente en comparación con los sujetos sanos. El análisis regional de atrofia cerebral mostró que los cambios en la corteza insular y la corteza frontal medial están significativamente relacionados con la variabilidad del rendimiento sociocognitivo entre los pacientes. Conclusiones: En el presente estudio, la cognición social sólo se correlacionó con el deterioro de la fluencia verbal, a pesar de que estudios previos han reportado su vinculación con la memoria y funciones ejecutivas. La identificación de correlatos estructurales específicos apoya la comprensión de este fenómeno como una fuente independiente de discapacidad cognitiva en estos pacientes.

Aspectos neuroquirúrgicos de las mucopolisacaridosis / Neurosurgical aspects of mucopolysaccharidoses

Mucopolisacaridosis es una rara enfermedad que afecta al metabolismo de los mucopolisacaridos debida a la ausencia o deficiencia de las enzimas encargadas de su síntesis lo que produce depósitos de aminoglucósidos en casi todos los tejidos del organismo. De acuerdo a la enzima faltante se clasifican los distintos tipos de la enfermedad, siendo más frecuente el Tipo I con sus tres variantes: Hurler, Hurler-Sheie y Sheie de distinta gravedad y tratamiento. Al nacimiento el niño no presenta síntomas, éstos van apareciendo a partir del año de vida: retardo físico y mental, múltiples deformidades esqueléticas, hepatoesplenomegalia, sordera, opacidades corneanas, entre otras. La intervención neuroquirúrgica en esta entidad es en dos patologías: la hidrocefalia, que al no presentar los signos clásicos de hipertensión endocraneana puede ser confundida con atrofia y la compresión medular cervical por los depósitos de mucopolisacáridos en vértebras, ligamentos y leptomeninges.

Mucopolysaccharidosis is a rare illness that involves the metabolism of mucopolysaccharides, that due to the absence or deficiency of corresponding enzymes, accumulate in almost all the tissues of the body. According to which enzyme is missing, different types of the disease have been identified; the most frequent being Type I with its three variants: Hurler, Hurler-Sheie, and Sheie. Symptoms of this disorder progress and range from mental and physical retardation, multiple skeletal deformities, hepatosplenomegaly, deafness, and corneal opacities among others. Children affected usually appear normal at birth and the slowness in their development may be the first evidence of the disorder whose progression is downhill. Neurosurgical intervention occurs in two pathologies: hydrocephalus that does not show signs of intracranial hypertension and can be confused with atrophy, and cervical cord compression due to storage of mucopolysaccharides in vertebrae, ligaments, and leptomeninges.

Complicaciones neurológicas en un niño con neumonía por influenza: reporte de caso clínico / Neurological complications in a child with influenza pneumonia: clinical case report

RESUMEN La influenza es una enfermedad endémica transmisible, en Ecuador los subtipos de virus que circulan son A(H1N1), A (H3N2) y tipo B. Existen dos picos de circulación del virus que fueron registrados en Ecuador en los años 2016 y 2018, siendo éste último el de mayor incidencia. Las complicaciones neurológicas asociadas a la influenza han sido descritas de manera poco frecuente, representando el 10% de los niños afectados y los reportes que existen la mayoría son de niños y adultos jóvenes. En el presente artículo se reporta un caso ocurrido en el 2018 de un lactante mayor con cuadro de neumonía por Influenza A/H3, Influenza A/pan1, Influenza A/pan2, virus Parainfluenza 1, complicada con derrame pleural izquierdo y atrofia cerebral y microhemorragias cerebrales.Palabras claves: Influenza; neumonía; neurología; derrame pleural; atrofia cerebral; hemorragia cerebral; informes de caso

BSTRACT Influenza is a transmissible endemic disease, in Ecuador the virus subtypes that circulate are A (H1N1), A (H3N2) and type B. There are two peaks of virus circulation that were registered in Ecuador in 2016 and 2018, the latter being the one with the highest incidence. The neurological complications associated with influenza have been described infrequently, representing 10% of the affected children and the reports that there are the majority are of children and young adults. This article presents a case in 2018 of an older infant with Influenza A / H3 pneumonia, Influenza A / pan1, Influenza A / pan2, Parainfluenza virus 1, complicated with left pleural effusion and cerebral atrophy and cerebral microhemorrhages.Keywords: Influenza; pneumonia; neurology; pleural effusion; Brain atrophy; cerebral haemorrhage; case reports.Correspondencia: Paúl MoscosoTeléfonos: (593) 987 038 828e-mail: paulmoscoso@hotmail.comIDs OrcidPaúl Moscoso: https://orcid.org/0000-0001-9018-7611Dayana Navarro: https://orcid.org/0000-0001-8601-9965Nicolás Espinosa:https://orcid.org/0000-0001-9825-0136

Xantomatosis cerebrotendinosa: Reporte de un caso en el Perú: Evaluación clínica integral y estudios de neuroimágenes / Cerebrotendinous Xanthomatosis: Report of a case in Peru: Comprehensive clinical evaluation and neuroimaging studies

Se presenta el caso de una paciente de 73 años de edad que, a los 30 años aproximadamente, comenzó a quejarse de dolor al caminar, localizando la molestia a nivel de las regiones aquilianas, con subsecuente aumento de volumen; al paso del tiempo, estas molestias la obligaron a efectuar consulta médica. Los análisis de laboratorio mostraron severa dislipidemia mixta. Al lado de información de significativa declinación cognitiva, provista por familiares (vgr., (i.e., olvidos frecuentes, desorientación, atención disminuida, concentración pobre), hubo evidencia de ánimo fluctuante, labilidad emocional, crisis ansiosas evolucionando hacia ataques de pánico. El test minicognitivo de Folstein, mostró severo estado demencial y en el examen neurológico se constataron ataxia cerebelosa y signos de piramidalismo parcial. El examen oftalmológico puso en evidencia xantelasmas, cataratas y un denso arco senil. El estudio del cerebro con resonancia magnética (RM) mostró el daño encefálico y signos sugestivos de depósitos del colastenol en el SNC. La presencia de xantomas , los hallazgos oftalmológicos, la demencia definidamente progresiva y la ataxia cerebelosa fueron hallazgos clínicos que permitieron establecer el diagnóstico de xantomatosis cerebrotendinosa.

The case of a 73 years-old woman that, since approximately the age of 30 years started to complain of pain when walking, is presented. The symptom was mainly located in the acchillean regions which, as time advanced, showed gradual volume increase and, finally, forced her to seek medical evaluation. Accompanying relatives reported a several years’ history of gradually increasing cognitive difficulties (i.e., forgetfulness, disorientation, poor attention and concentration), fluctuating mood (from periods of good humor switching to sudden episodes of sadness and crying spells), emotional lability and anxiety crises evolving into brief panic attacks. The Mini-cognitive Fenton Test confirmed severe dementia and the neurological evaluation showed cerebellar ataxia and partial pyramidalism. The ophthalmological examination revealed xanthelasmas, cataracts and dense arcus senilis. Xanthomas were detected in the Achillean tendons of both lower extremities. Auxiliary laboratory and densitometric tests demonstrated mixed dyslipidemia and dorsal-lumbar osteoporosis, respectively, and magnetic resonance imaging of the brain (RMC) confirmed SNC damage and suggested deposits of cholestenol, thus confirming the diagnosis of Cerebroitendinous Xanthomatosis.

El raro caso de una joven con colitis ulcerativa crónica inespecífica grave y atrofia cerebral / The rare case of a young girl with ulcerative colitis and cerebral atrophy

Resumen La enfermedad inflamatoria intestinal es un grupo de enfermedades caracterizadas por inflamación crónica gastrointestinal y en ocasiones con repercusión extraintestinal. Las manifestaciones neurológicas y psiquiátricas corresponden a menos de 3%. Se comunica el caso de una mujer joven con colitis ulcerativa y atrofia cerebral como inicio.

Abstract Inflammatory bowel disease is a group of diseases characterized by chronic gastrointestinal inflammation and occasionally with extraintestinal repercussion. The neurological and psychiatric manifestations correspond to less than 3%. This paper reports the case of a young woman with ulcerative colitis and cerebral atrophy as debut.

Age-related cerebral ventricular volume changes in healthy elderly humans / Alterações volumétricas dos ventrículos cerebrais relacionadas a idade em idosos saudáveis

Introduction: Normal aging is associated with morphological alte-rations in brain. Ventricular system is located deep inside brain and reflect the overall process of parenchymal atrophy. Once neurode-generative disorders course with more prominent dilatation of brain ventricles, to establish normative volumetric parameters from Brazi-lian healthy old individuals is necessary, and it may be an additional tool on differentiation from the normal to pathological. Objective: To investigate brain ventricular volume changes in Brazilian healthy el-derly people. Methods: Transversal study using magnetic resonance imaging (1.5T) of the brain from 21 elderly healthy volunteers (67±6 years old). Data were assessed with manual segmentation techni-que. Regions of interest were the brain ventricles and intracranial volumes. Old (60-69 years old, 15 women) and Older (>69 years old) groups were created for analysis. Results: Volume of all ventricular compartments significantly increased (p<.001) with age, with excep-tion of the fourth ventricle. The third and lateral ventricles increased between groups 2.1- and 2.8-fold, respectively. Mean total ventricular volume was 1.2±.4% of intracranial volume in Old and 3.2±1.8% in Ol-der group (p<.001), which represents 15±6ml and 40±24ml (p=.001), respectively. We observed a moderate to strong positive correlation between ventricular volume and age, with the best correlation in the third ventricle (r=.710). Total intracranial volume diminished with age, but without statistical significance. Conclusions: Brain ventricles vo-lume increased significantly with age in healthy old individuals, with exception of the fourth ventricle. (AU)

Introdução: O envelhecimento normal está associado a alterações morfológicas do cérebro. O sistema ventricular está localizado pro-fundamente no encéfalo e reflete o processo global de atrofia do pa-rênquima. Uma vez que doenças neurodegenerativas cursam com dilatação mais proeminente dos ventrículos cerebrais, estabelecer parâmetros volumétricos de normalidade em nossa população idosa saudável se faz necessário, podendo ser uma ferramenta a mais para diferenciar o normal do patológico. Objetivo: Investigar alterações volumétricas dos ventrículos cerebrais em brasileiros idosos sau-dáveis. Métodos: Estudo transversal com imagens de ressonância magnética (1,5T) do encéfalo de 21 idosos saudáveis (68±6 anos, 15 mulheres). Os dados foram examinados por técnicas de segmenta-ção manual. As regiões de interesse foram os ventrículos cerebrais e o volume intracraniano. Criamos os subgrupos Idosos (60-69 anos) e Mais idosos (>69 anos) para a análise. Resultados: O volume de todos os ventrículos aumentou com a idade (p<0,001), com exceção do quarto ventrículo. O terceiro e os ventrículos laterais aumentaram 2,1 e 2,8 vezes, respectivamente, entre os grupos. O volume ventri-cular médio foi de 1,2±0,4% do volume intracraniano nos Idosos e de 3,2±1,8% nos mais idosos, o que representa 15±6ml e 40±24ml, respectivamente. Observamos correlação positiva de moderada a forte entre volume ventricular e idade, com a melhor correlação no terceiro ventrículo (r=0,710). O volume intracraniano diminui com a idade, sem significância estatística. Conclusão: os ventrículos cere-brais aumentam significativamente com o envelhecimento em idosos saudáveis, exceto o quarto ventrículo. (AU)

Brain atrophy in multiple sclerosis: therapeutic, cognitive and clinical impact / Atrofia cerebral en esclerosis múltiple: impacto clínico, cognitivo y terapéutico

ABSTRACT Multiple sclerosis (MS) was always considered as a white matter inflammatory disease. Today, there is an important body of evidence that supports the hypothesis that gray matter involvement and the neurodegenerative mechanism are at least partially independent from inflammation. Gray matter atrophy develops faster than white matter atrophy, and predominates in the initial stages of the disease. The neurodegenerative mechanism creates permanent damage and correlates with physical and cognitive disability. In this review we describe the current available evidence regarding brain atrophy and its consequence in MS patients.

RESUMEN La esclerosis múltiple (EM) fue considerada históricamente como una enfermedad inflamatoria de la sustancia blanca. Hoy en día hay mucha evidencia que apoya, además, el compromiso de la sustancia gris y los mecanismos neurodegenerativos, que son al menos parcialmente independientes de la inflamación. La atrofia de la sustancia gris se desarrolla más rápido que la atrofia de la sustancia blanca y predomina en las etapas iniciales de la enfermedad. El mecanismo neurodegenerativo, crea un daño permanente y se correlacionaría con la discapacidad física y cognitiva del paciente. En esta revisión, se describe la evidencia disponible actual con respecto a la atrofia cerebral y su consecuencia en los pacientes con EM.

Brain atrophy at onset and physical disability in multiple sclerosis / Atrofia cerebral al inicio de la enfermedad y discapacidad fisica en esclerosis multiple

The aim of this study was to investigate if brain atrophy in multiple sclerosis (MS) patients during the disease onset predicts long term disability. METHODS: MS patients with follow-up time of at least 7 years from disease onset and with baseline and second magnetic resonance 12 months later were included to measure brain atrophy. Expanded Disability Status Scale (EDSS) was categorized in three groups, EDSS=0, EDSS=1 and 2.5 and EDSS>2.5, and used as disability measure. RESULTS: Twenty-six patients were included. Mean atrophy during the first year in patients that reached an EDSS≥3 was -0.76±0.45 %, in patients with an EDSS between 1 and 2.5 was -0.59±0.56, while in patients with an EDSS of 0 it was -0.38±0.42 (p=0.003). DISCUSSION: Brain atrophy rates during the first year of disease were predictive of disease progression in our population.

El objetivo fue evaluar en pacientes con esclerosis múltiple (EM) si la atrofia durante el primer año de iniciada la enfermedad predecía la discapacidad física a largo plazo. MÉTODOS: Pacientes con EM seguidos al menos durante 7 años del inicio de la enfermedad y con una resonancia magnetica al inicio y una segunda a los 12 meses de la inicial fueron incluidos para evaluar la atrofia cerebral. El Expanded Disability Status Scale (EDSS) fue categorizado en tres grupos, EDSS=0, EDSS=1 y 2.5 y EDSS>2.5, y usado como medida de la discapacidad. RESULTADOS: Veintiséis pacientes fueran incluidos. El porcentaje de atrofia durante el primer año de iniciada la enfermedad en los pacientes que alcanzaron un EDSS≥3 fue de -0.76±0.45%, de -0.59 ±0.56 en pacientes con EDSS entre 1 y 2.5; de -0.38±0.42 en pacientes con EDSS de 0 (p=0,003). DISCUSIÓN: La tasa de atrofia cerebral durante el primer año de la esclerosis múltiple fue predictora de progresión de la discapacidad.

Clinical assessment, neuroimaging and immunomarkers in Chagas disease study (CLINICS): rationale, study design and preliminary findings / Avaliação clínica, neuroimagem e imunomarcadores na doença de Chagas (CLINICS): fundamentação teórica, desenho do estudo e resultados preliminares

Chagas disease (CD) is an important cause of cardiomyopathy and stroke in Brazil. Brain infarcts and atrophy seem to occur independently of cardiomyopathy severity and cognitive impairment is under studied. Objective: Compare the prevalence of brain magnetic resonance imaging abnormalities between patients with or without CD; determine if inflammatory biomarkers are increased in CD; and determine the efficacy of aspirin in reducing the rate of microembolization in these patients. Methods: 500 consecutive patients with heart failure will undergo a structured cognitive evaluation, biomarker collection and search for microembolic signals on transcranial Doppler. The first 90 patients are described, evaluated with cognitive tests and brain magnetic resonance imaging to measure N-acetyl aspartate (NAA), choline (Cho), myo-inositol (MI) and creatine (Cr). Results: Mean age was 55±11 years, 51% female, 38 (42%) with CD. Mean NAA/Crratio was lower in patients with CD as compared to other cardiomyopathies. Long-term memory and clock-drawing test were also significantly worse in CD patients. In the multivariable analysis correcting for ejection fraction, age, sex and educational level, reduced NAA/Cr (p=0.006) and cognitive dysfunction (long-term memory, p=0.023; clock-drawing test, p=0.015)remained associated with CD. Conclusion: In this preliminary sample, CD was associated with cognitive impairment and decreased NAA/Cr independently of cardiac function or educational level.

A doença de Chagas (DC) é causa importante de cardiomiopatia e acidente vascular cerebral no Brasil. Os infartos e atrofia cerebral na DC parecem ocorrer independente da gravidade da cardiomiopatia, sendo o comprometimento cognitivo pouco estudado. Objetivo: Determinar a prevalência de alterações na ressonância magnética entre chagásicos e não chagásicos; determinar se os níveis de marcadores inflamatórios estão aumentados na DC e determinar a eficácia da aspirina em reduzir a taxa de microembolização nestes pacientes. Métodos: Quinhentos pacientes consecutivos com diagnóstico de insuficiência cardíaca serão submetidos a uma avaliação cognitiva estruturada, coleta de biomarcadores e pesquisa de sinais de microembolia por Doppler transcraniano. Os primeiros 90 pacientes são descritos, avaliados por testes cognitivos e ressonância magnética cerebral, com medida de N-acetil aspartato (NAA), colina (Cho), mioinositol (MI)e creatina (Cr). Resultados: A idade média foi de 55±11 anos, 51% eram do sexo feminino, 38 (42%) tinha DC. A médiada relação NAA/Cr foi mais baixa em pacientes com DC quando comparada com outras miocardiopatias. O desempenho nos testes de memória de longo prazo e desenho do relógio foi significativamente pior nos portadores de DC. Na análise multivariada, corrigindo para fração de ejeção, idade, gênero e nível educacional, redução da relação NAA/Cr (p=0.006) e disfunção cognitiva (memória de longo prazo, p=0.023; teste do desenho do relógio, p=0.015) permaneceram associados a DC. Conclusão: Nesta amostra preliminar, a doença de Chagas esteve associada a disfunção cognitiva e redução dos níveis de NAA/Cr, independente da função cardíaca e nível educacional.

Oligoclonal bands in the cerebrospinal fluid and increased brain atrophy in early stages of relapsing-remitting multiple sclerosis / Bandas oligoclonales en líquido cefalorraquídeo y incremento de la atrofia cerebral en estadios tempranos de pacientes con esclerosis múltiple forma recaída-remisión

OBJECTIVE: To determine if the presence of oligoclonal bands (OB) at early stages of multiple sclerosis was associated with higher brain atrophy, when compared with patients without OB. METHODS: Relapsing-remitting multiple sclerosis (RRMS) patients with less than two years of disease onset and OB detection in cerebrospinal fluid (CSF) were included. SIENAX was used for total brain volume (TBV), gray matter volume (GMV), and white matter volume (WMV). RESULTS: Forty patients were included, 29 had positive IgG-OB. No differences were found between positive and negative patients in gender, expanded disability status scale (EDSS), treatment received, and T2/T1 lesion load. TBV in positive IgG-OB patients was 1.5 mm³ x 10(6) compared with 1.64 mm³ x 10(6) in the negative ones (p=0.02). GMV was 0.51 mm³ x 10(6) in positive IgG-OB compared with 0.62 mm³ x 10(6) in negative ones (p=0.002). No differences in WMV (p=0.09) were seen. CONCLUSIONS: IgG-OB in the CSF was related to neurodegeneration magnetic resonance (MR) markers in early RRMS.

OBJETIVO: Evaluar si la presencia de bandas oligoclonales (BO) en líquido cefalorraquídeo (LCR) de pacientes con esclerosis múltiple recaídaremisión (EMRR) se asociaba con mayor atrofia cerebral al inicio de la enfermedad. MÉTODOS: Pacientes con EMRR con menos que dos años del inicio de la enfermedad y en quiénes se realizó la búsqueda de IgG-BO en LCR fueron incluidos. SIENAX fue usado para la medición del volumen cerebral total (VCT), volumen de substancia gris (VSG) y volumen de sustancia blanca (VSB). RESULTADOS: Cuarenta pacientes fueron incluidos, 29 tenían IgG-BO positivo. No fueron encontradas diferencias entre pacientes positivos y negativos en: género, expanded disability status scale (EDSS), tratamiento recibido y carga lesional en resonancia magnética. El VCT en pacientes IgG-BO positivos fue de 1,5 mm³ x 10(6) versus 1,64 mm³ x 10(6) en BO negativo (p=0,02). El VSG fue 0,51 mm³ x 10(6) BO positivo versus 0,62 mm³ x 10(6) BO negativo (p=0,002). No fueron encontradas diferencias en VSB (p=0,09). CONCLUSIONES: La presencia de IgG-BO en el LCR se asoció con signos de neurodegeneración temprana en este estudio.

Early and severe cognitive impairment in multiple sclerosis / Comprometimento cognitivo precoce e grave na esclerose múltipla

To report on four new cases of severe cognitive impairment in the early stages of multiple sclerosis(MS) and to review data on the subject since few cases have been reported world wide. Methods: Retrospective evaluationof medical records of patients with severe cognitive impairment within the first five years of MS diagnosis. Results on neuropsychological tests and magnetic resonance imaging (MRI) were disclosed. Results: Four patients from different Brazilian neurological departments in Brazil were evaluated, all presenting with severe cognitive dysfunction classified as rapidly developing dementia. MRI images showed severe brain atrophy and basal ganglia lesions in all patients. Conclusions: Although rare, severe cognitive impairment in MS represents an important disability and may ultimately constitute anotherform of the disease.

Relatar quatro novos casos de grave comprometimento cognitivo nas fases iniciais da esclerose múltipla(EM). Revisão da literatura no tema, uma vez que muito poucos casos foram descritos no mundo. Métodos: Avaliação retrospectiva dos prontuários de pacientes com grave comprometimento cognitivo nos cinco primeiros anos de EM. Osresultados de testes neuropsicológicos e de imagens de ressonância magnética (RNM) são apresentados. Resultados: Quatro pacientes de diferentes departamentos de neurologia brasileiros foram avaliados, todos apresentando disfunçãocognitiva muito grave que pode ser classificada como demência de rápida evolução. As imagens na RNM mostraram graveatrofia cerebral e lesões em gânglios da base em todas as pacientes. Conclusões: Embora muito rara, a alteração cognitivagrave na EM representa uma incapacidade importante e pode, em última análise, ser outra forma da doença.

Bilateral cortical atrophy after severe brain trauma and extradural homatoma

We report the case of a severe head injured 43-year old male patient with a large extradural hematoma, Glasgow Coma Scale 3 and dilated fixed pupils. Patient was promptly submitted to surgical evacuation of the lesion, but remained in persistent vegetative state in the post-operative time. Head computed tomography scans performed before surgery, and at early and late post-operative periods comparatively revealed extreme bilateral cortical atrophy. Late consequences of severe head trauma drastically affect the prognosis of patients, being its prevention, and neuroprotection against secondary injury still a therapeutical challenge for neurosurgeons.

Relatamos o caso de um paciente de 43 anos, com traumatismo cranioencefálico grave, com grande hematoma extradural, Escala de Coma de Glasgow 3 e pupilas fixas e dilatadas. O paciente foi prontamente submetido à evacuação cirúrgica da lesão mas permaneceu em estado vegetativo persistente no período pós-operatório. As TC de crânio realizadas antes da cirurgia e nos períodos pós-operatórios precoce e tardio revelaram comparativamente extrema atrofia cerebral bilateral. As conseqüências tardias do traumatismo craniano grave afetam drasticamente o prognóstico dos pacientes, sendo sua prevenção, e a neuroproteção contra a injúria secundária ainda um desafio terapêutico para os neurocirurgiões.