Autoimmune encephalitis. A review Encefalitis autoinmunes: Criterios diagnósticos y pautas terapéuticas

© 2018, Sociedad Medica de Santiago. All Rights reserved.Autoimmune encephalitis are one of the emergent causes of subacute changes in the level of consciousness, behavior, cognitive impairment and seizures, mainly in young people. They are a consequence of inflammation or dysfunction of parts of the brain caused by antibodies against specific brain antigens, usually located in the limbic system, resulting in clinical presentation as a limbic encephalitis. The objectives of this article are to show the clinical presentation, complementary studies and treatment of this entity, considering that the patient´s prognostic depends on a high level of clinical suspicion, and on an early initiation of immunosuppressive therapy. We did a nonsystematic review of the literature on autoimmune encephalitis between 2005 and 2017. We conclude that the prevalence of autoimmune encephalitis is increasing, even surpassing infectious causes of encephalitis in developed countries. Clinical presentation in

Dementia Validation of the Spanish-language version of the Montreal Cognitive Assessment testin adults older than 60 year

Introducción: Existen pocas validaciones de la versión en español de la prueba Montreal Cognitive Assessment (MoCA-S) en Latinoamérica. Objetivo: Evaluar las propiedades psicométricas y la validez discriminativa del MoCA-S en adultos mayores de Santiago de Chile. Métodos: Ciento setenta y dos individuos agrupados según diagnóstico clínico basado en Clinical Dementia Rating (CDR) en: deterioro cognitivo leve tipo amnésico (DCL-a, n = 24), DCL noamnésico (DCL-na, n = 24), demencia leve (n = 20) y 104 cognitivamente sanos fueron evaluados con el MoCA-S y Mini-Mental test de Folstein (MMSE) como prueba de contraste, para determinar la validez discriminativa del MoCA-S. Resultados: Los promedios ± desviación estándar de edad y escolaridad fueron 73 ± 6 y 11 ± 4, respectivamente, sin diferencias significativas entre los grupos. La consistencia interna fue buena ( de Cronbach 0,772), la fiabilidad interevaluador muy buena (coeficiente de correlación de Spearman 0,846 [p < 0,01]) y la fiabilidad intraevaluador (test-retest) fue 0,922 bilateral (p < 0,001). La prueba fue eficaz y válida para la detección del DCL-a (ABC = 0,903) y demencia leve (ABC = 0,957); menos eficaz en DCL no-a (ABC = 0,629). El punto de corte de mejor rendimiento para DCL-a fue < 21 y para demencia leve < 20; sensibilidad/especificidad de 75/82% y 90/86%, respectivamente. La escolaridad mostró una importante influencia en el puntaje, por ello se adicionaron 2 puntos para escolaridad < 8 años y un punto para escolaridad entre 8 y 12 años (MoCA-S1-2). El MoCA-S1-2 fue significativamente más discriminativo que el MMSE para diferenciar DCL-a y demencia. Introducción: Existen pocas validaciones de la versión en español de la prueba Montreal Cognitive Assessment (MoCA-S) en Latinoamérica. Objetivo: Evaluar las propiedades psicométricas y la validez discriminativa del MoCA-S en adultos mayores de Santiago de Chile. Métodos: Ciento setenta y dos individuos agrupados según diagnóstico clínico basado en Clinical Dementia Rating (CDR) en: deterioro cognitivo leve tipo amnésico (DCL-a, n = 24), DCL noamnésico (DCL-na, n = 24), demencia leve (n = 20) y 104 cognitivamente sanos fueron evaluados con el MoCA-S y Mini-Mental test de Folstein (MMSE) como prueba de contraste, para determinar la validez discriminativa del MoCA-S. Resultados: Los promedios ± desviación estándar de edad y escolaridad fueron 73 ± 6 y 11 ± 4, respectivamente, sin diferencias significativas entre los grupos. La consistencia interna fue buena ( de Cronbach 0,772), la fiabilidad interevaluador muy buena (coeficiente de correlación de Spearman 0,846 [p < 0,01]) y la fiabilidad intraevaluador (test-retest) fue 0,922 bilateral (p < 0,001). La prueba fue eficaz y válida para la detección del DCL-a (ABC = 0,903) y demencia leve (ABC = 0,957); menos eficaz en DCL no-a (ABC = 0,629). El punto de corte de mejor rendimiento para DCL-a fue < 21 y para demencia leve < 20; sensibilidad/especificidad de 75/82% y 90/86%, respectivamente. La escolaridad mostró una importante influencia en el puntaje, por ello se adicionaron 2 puntos para escolaridad < 8 años y un punto para escolaridad entre 8 y 12 años (MoCA-S1-2). El MoCA-S1-2 fue significativamente más discriminativo que el MMSE para diferenciar DCL-a y demencia. Conclusiones: El MoCA-S1-2 es una prueba breve, de fácil administración y útil para el diag-nóstico de DCL-a y demencia leve.Introduction: Few studies have validated the Spanish-language version of the Montreal Cogni-tive Assessment (MoCA-S) test in Latin American populations.Objetive: To evaluate the psychometric properties and discriminant validity of the MoCA-S inelderly patients in Santiago de Chile.Methods: 172 individuals were grouped according to their clinical diagnosis based on the Clini-cal Dementia Rating (CDR) scale as follows: amnestic mild cognitive impairment (aMCI; n ± 24),non-amnestic MCI (naMCI; n ± 24), mild dementia (n ± 20), and cognitively normal (n ± 104). Par-ticipants were evaluated with both the MoCA-S and the Mini—Mental State Examination (MMSE)to determine the discriminant validity of the MoCA-S.Results: Mean age and years of schooling were 73 ± 6 and 11 ± 4 years, respectively, withno significant intergroup differences. The MoCA-S displayed good internal consistency (Cron-bach’s : 0.772), high inter-rater reliability (Spearman correlation coefficient: 0.846; P<.01),and high intra-rater reliability (test-retest reliability coefficient: 0.922; P<.001). The MoCA-Swas found to be an effective and valid test for detecting aMCI (AUC ± 0.903) and mild demen-tia (AUC ± 0.957); its effectiveness for detecting naMCI was lower (AUC ± 0.629). The optimalcut-off points for aMCI and mild dementia were < 21 and < 20, respectively, with sensitivity andspecificity rates of 75% and 82% for aMCI and 90% and 86% for mild dementia. The level of edu-cation had a great impact on scores: as a result, 2 points were added for patients with less than8 years of schooling and one point for patients with 8-12 years of schooling (MoCA-S1-2). TheMoCA-S1-2 showed significantly greater discriminant validity than the MMSE for differentiatingaMCI from dementia.Conclusions: The MoCA-S1-2 is a short, easy-to-use, and useful test for diagnosing aMCI andmild dementia

Autoimmune encephalitis. A review

Autoimmune encephalitis are one of the emergent causes of subacute changes in the level of consciousness, behavior, cognitive impairment and seizures, mainly in young people. They are a consequence of inflammation or dysfunction of parts of the brain caused by antibodies against specific brain antigens, usually located in the limbic system, resulting in clinical presentation as a limbic encephalitis. The objectives of this article are to show the clinical presentation, complementary studies and treatment of this entity, considering that the patient's prognostic depends on a high level of clinical suspicion, and on an early initiation of immunosuppressive therapy. We did a nonsystematic review of the literature on autoimmune encephalitis between 2005 and 2017. We conclude that the prevalence of autoimmune encephalitis is increasing, even surpassing infectious causes of encephalitis in developed countries. Clinical presentation includes sub-acute cognitive and behavioral impairment, with or without alterations in consciousness and seizures. Fever and inflammation of the cerebrospinal fluid are less common than in the infectious causes but psychiatric symptoms are more frequent. There are specific clinical presentations according to the particular type of antigen/antibody present, which also determines the association with cancer, constituting a paraneoplastic syndrome only in some cases. Immunosuppressive therapy has been standardized in steps, and should be initiated early to improve prognosis

Comparison of the Psychometric Properties of the "Word" and "Picture" Versions of the Free and Cued Selective Reminding Test in a Spanish-Speaking Cohort of Patients with Mild Alzheimer's Disease and Cognitively Healthy Controls

Artículo de publicación ISIThe aim of this study was to compare the psychometric properties of the "Word" and "Picture" versions of the Spanish FCSRT across the same sample of mild Alzheimer disease (AD) patients and controls. Mild AD patients (N = 50, 27 CDR = 0.5; 23 CDR = 1) and controls (N = 42, CDR = 0) were assessed with an extensive clinical and neuropsychological evaluation. Psychometric characteristics for both versions of the FCSRT were compared. Free recall (FR) and total recall (TR) across both versions of the FCSRT showed areas under the curve > 0.9 and did not significantly differ between them. The scores of both versions were well correlated, although the scores for the Picture version were greater than those for the Word version, particularly for the TR scores of the mild AD group. Both versions of the FCSRT showed an appropriate accuracy to distinguish mild AD patients and controls. Visual cues were easier to recall than verbal cues, especially in the memory impaired patients.Fondo Nacional de Desarrollo Cientifico y Tecnologico (FONDECYT), Comision Nacional de Investigacion Cientifica y Tecnologica, Gobierno de Chile, FONDAP, Basal Funds for Excellence Centers from the Associative Research Program, Comision Nacional de Investigacion Cientifica y Tecnologica (CONICYT), Gobierno de Chile

COGNITIVE PERFORMANCE IN PATIENTS WITH CORONARY HEART DISEASE AND CARDIOVASCULAR RISK FACTORS IN CHILE

Introducción. La presencia de factores de riesgo cardiovascular (FRCV) se relaciona con una peor evolución cognitiva a largo plazo. La cardiopatía coronaria (CC) se ha asociado inconstantemente a mala evolución cognitiva. Objetivos. Se trata de evaluar el rendimiento cognitivo de pacientes chilenos con distintos grados de FRCV, con y sin CC. Sujetos y métodos. Es un estudio transversal de evaluación cognitiva en tres grupos de pacientes mayores de 60 años sin antecedentes de ataques cerebrovasculares: con CC y FRCV elevados (CC-FRCVE) (n = 62), con FRCV elevados sin CC (FRCVE) (n = 57), y sin antecedentes de hipertensión arterial (HTA), diabetes ni CC o ‘no hipertenso no diabético’ (NHND) (n = 25). Resultados. En la totalidad de la muestra los factores asociados significativamente a deterioro cognitivo fueron: mayor edad, menor escolaridad y ser hombre. No hubo diferencias significativas en la edad ni en la proporción de pacientes con deterioro cognitivo entre los tres grupos; los NHND tenían significativamente mayor escolaridad que los otros grupos. Los CC-FRCVE tuvieron mayor proporción de hombres y mayor proporción de enfermedad arterial oclusiva periférica y dislipidemia que los otros grupos. Los FRCVE tuvieron significativamente mayor cantidad de años con HTA que los otros grupos. En las escalas cognitivas y de depresión, no se encontraron diferencias estadísticamente significativas en el rendimiento entre los tres grupos después de ajustar los resultados por escolaridad y sexo, y existió significativamente mayor dependencia funcional en el grupo coronario. Conclusiones. La CC en pacientes sin antecedentes de enfermedad cerebrovascular, en forma aislada, no se asocia a peor rendimiento cognitivo global, pero sí a mayor daño vascular sistémico y mayor dependencia funcional

Insula and Amygdala Atrophy Are Associated With Functional Impairment in Subjects With Presbycusis

Hearing loss is an important risk factor for dementia. However, the mechanisms that relate these disorders are still unknown. As a proxy of this relationship, we studied the structural brain changes associated with functional impairment in activities of daily living in subjects with age related hearing loss, or presbycusis. One hundred eleven independent, non-demented subjects older than 65 years recruited in the ANDES cohort were evaluated using a combined approach including (i) audiological tests: hearing thresholds and cochlear function measured by pure tone averages and the distortion product otoacoustic emissions respectively; (ii) behavioral variables: cognitive, neuropsychiatric, and functional impairment in activities of daily living measured by validated questionnaires; and (iii) structural brain imaging-assessed by magnetic resonance imaging at 3 Tesla. The mean age of the recruited subjects (69 females) was 73.95 +/- 5.47 years (mean +/- SD) with an average educational level of 9.44 +/- 4.2 years of schooling. According to the audiometric hearing thresholds and presence of otoacoustic emissions, we studied three groups: controls with normal hearing (n = 36), presbycusis with preserved cochlear function (n = 33), and presbycusis with cochlear dysfunction (n = 38). We found a significant association (R-D(2) = 0.17) between the number of detected otoacoustic emissions and apathy symptoms. The presbycusis with cochlear dysfunction group had worse performance than controls in global cognition, language and executive functions, and severe apathy symptoms than the other groups. The neuropsychiatric symptoms and language deficits were the main determinants of functional impairment in both groups of subjects with presbycusis. Atrophy of insula, amygdala, and other temporal areas were related with functional impairment, apathy, and language deficits in the presbycusis with cochlear dysfunction group. We conclude that (i) the neuropsychiatric symptoms had a major effect on functional loss in subjects with presbycusis, (ii) cochlear dysfunction is relevant for the association between hearing loss and behavioral impairment, and (iii) atrophy of the insula and amygdala among other temporal areas are related with hearing loss and behavioral impairment.Comisión Nacional de Investigación Cientifica y Tecnológica (CONICYT) CONICYT FONDECYT 1161155 Comisión Nacional de Investigación Cientifica y Tecnológica (CONICYT) CONICYT PIA/ANILLOS ACT1403 CONICYT BASAL FB008 Proyecto ICM P09015F Fundación Guillermo Puelm

Reduced suprathreshold auditory nerve responses are associated with slower processing speed and thinner temporal and parietal cortex in presbycusis

Epidemiological evidence shows an association between hearing loss and dementia in elderly people. However, the mechanisms that connect hearing impairments and cognitive decline are still unknown. Here we propose that a suprathreshold auditory-nerve impairment is associated with cognitive decline and brain atrophy. Methods: audiological, neuropsychological, and brain structural 3-Tesla MRI data were obtained from elders with different levels of hearing loss recruited in the ANDES cohort. The amplitude of waves I (auditory nerve) and V (midbrain) from auditory brainstem responses were measured at 80 dB nHL. We also calculated the ratio between wave V and I as a proxy of suprathreshold brainstem function. Results: we included a total of 101 subjects (age: 73.5 +/- 5.2 years (mean +/- SD), mean education: 9.5 +/- 4.2 years, and mean audiogram thresholds (0.5-4 kHz): 25.5 +/- 12.0 dB HL). We obtained reliable suprathreshold waves V in all subjects (n = 101), while replicable waves I were obtained in 92 subjects (91.1%). Partial Spearman correlations (corrected by age, gender, education and hearing thresholds) showed that reduced suprathreshold wave I responses were associated with thinner temporal and parietal cortices, and with slower processing speed as evidenced by the Trail-Making Test-A and digit symbol performance. Non-significant correlations were obtained between wave I amplitudes and other cognitive domains. Conclusions: These results evidence that reduced suprathreshold auditory nerve responses in presbycusis are associated with slower processing speed and brain structural changes in temporal and parietal regions

Tau platelets correlate with regional brain atrophy in patients with Alzheimer's disease

Background: Intracellular neurofibrillary tangles are part of the core pathology of Alzheimer's disease (AD), which are mainly composed of hyperphosphorylated tau protein. Objectives: The purpose of this study is to determine whether high molecular weight (HMW) or low molecular weight (LMW) tau protein levels, as well as the ratio HMW/LMW, present in platelets correlates with brain magnetic resonance imaging (MRI) structural changes in normal and cognitively impaired subjects. Methods: We examined 53 AD patients and 37 cognitively normal subjects recruited from two Memory Clinics at the Universidad de Chile. Tau levels in platelets were determined by immunoreactivity and the MRI scans were analyzed using voxel-based morphometry in 41 AD patients. Results: The HMW/LMW tau ratio was statistically different between controls and AD patients, and no associations were noted between HMW or LMW tau and MRI structures. In a multivariate analysis controlled for age and education level, the HMW/LMW tau ratio was associated with reduced volume in the left medial and right anterior cingulate gyri, right cerebellum, right thalamus (pulvinar), left frontal cortex, and right parahippocampal region. Conclusions: This exploratory study showed that HMW/LMW tau ratio is significantly higher in AD patients than control subjects, and that it is associated with specific brain regions atrophy. Determination of peripheral markers of AD pathology can help understanding the pathophysiology of neurodegeneration in ADCorfo INNOVA Chile 12IDL2-18218 International Center for Biomedicine (ICC) PIA-CONICYT Basal Funds for Centers of Excellence Project BF0003 NIH/NIA AG005133 CONICYT/FONDECYT/1100975 CONICYT/FONDECYT/1140423 CONICYT/FONDECYT/1110373 CONICYT/FONDAP/1515001