The reliability of neurobehavioral tests in a thai adult population / A confiabilidade dos testes neurocomportamentais em uma população adulta tailandesa

ABSTRACT Early detection of decline in neurobehavioral (NB) performance requires reliable methods of testing. Although NB tests have been shown to be consistent and reliable in Western countries, there has been limited research in Asian populations. Objective: The purpose of this study was to investigate the test-retest reliability of NB tests in a Thai adult population and examine the impact of demographic data on NB tests. The aspects of the tests chosen were memory, attention, hand-eye coordination, motor speed, and dexterity. Methods: The three NB tests used were digit span, Purdue Pegboard, and visual-motor integration. All three were administered to a population of 30 Thai adults. Results: The outcomes of all Pearson's correlation coefficient tests (r) were positive and greater than 0.60, and subtest-retest reliability correlation coefficients ranged from 0.63 (p<0.001) to 0.81 (p<0.001). Interestingly, the outcomes of all of these tests were not affected by demographic data, with the exception of the Purdue Pegboard test, in which performance on the preferred hand and both hands assessment was weakly associated with age (β=-0.09, p<0.001 and β=-0.08, p<0.05, respectively). Conclusions: NB tests have adequate reliability and are useful for the evaluation of clinical memory, attention, hand-eye coordination, motor speed, and dexterity in Thai adults. These tests were not affected by demographic data. However, further studies to measure the validity of the digit span, Purdue Pegboard, and visual-motor integration tests are needed.

RESUMO A detecção precoce do declínio no desempenho neurocomportamental (NC) requer métodos confiáveis de teste. Embora os testes NC tenham se mostrado consistentes e confiáveis em países ocidentais, as pesquisas em populações asiáticas ainda são limitadas. Objetivo: O objetivo deste estudo foi investigar a confiabilidade de teste-reteste dos testes NC em uma população adulta tailandesa e o impacto dos dados demográficos nos testes NC. Os aspectos dos testes escolhidos foram memória, atenção, coordenação óculo-manual, velocidade motora e destreza. Métodos: Os três testes RC utilizados foram o digit span, o Purdue Pegboard e a integração visomotora. Todos os três foram usados em uma população de 30 adultos tailandeses. Resultados: Os resultados de todos os testes de coeficiente de correlação de Pearson (r) foram positivos e superiores a 0,60, e os coeficientes de correlação de confiabilidade subteste-reteste variaram de 0,63 (p<0,001) a 0,81 (p<0,001). Curiosamente, os resultados de todos esses testes não foram afetados pelos dados demográficos, com exceção do teste Purdue Pegboard, no qual o desempenho na mão preferida e a avaliação de ambas as mãos foi fracamente associado à idade (β=-0,09, p<0,001 e β=-0,08, p<0,05, respectivamente). Conclusão: Os testes NC apresentam confiabilidade adequada e são úteis para avaliação da memória clínica, atenção, coordenação óculo-manual, velocidade motora e destreza em adultos tailandeses. Esses testes não foram afetados por dados demográficos. No entanto, são necessários mais estudos para medir a validade dos testes de digit span, Purdue Pegboard e IVM.

Correlation between cognitive function and living ability of older adult patients living in a mining community / 中国基层医药

Objective:To investigate the correlation between cognitive function and living ability of older adult patients living in a mining community.Methods:A total of 180 older adult patients living in a mining community who received treatment during July-October 2019 were included in this study. They were randomly divided into the low-age group (< 68 years old, n = 94) and the high-age group (≥ 68 years old, n = 86). Cognitive function and living ability were evaluated using the Mini-Mental State Examination (MMSE), The Montreal Cognitive Assessment (MoCA), and the Activity of Daily Living Scale (ADL). The relationship between cognitive function and living ability was investigated using hierarchical analysis and Pearson correlation analysis. Results:The proportions of older adult patients with abnormal cognitive function identified by the MMSE and MoCA were 39.4% and 66.0%, respectively in the low-age group, and they were 32.6% and 61.6%, respectively in the high-age group. The MoCA had a greater performance in identifying abnormal cognitive function in each group than the MMSE ( χ2 = 26.69, 10.18, both P < 0.001). There were no significant differences in proportions of older adult patients with abnormal cognitive function identified by the MMSE and MoCA between low-age and high-age groups ( χ2 = 0.90, 0.36, both P > 0.05). The proportion of older adult patients with abnormal living ability was not significantly different between low-age and high-age groups (4.3% vs. 10.5%, χ2 = 2.58, P > 0.05). Compared with patients negative for MMSE items, living ability and instrumental activity of daily living increased by 7.0% and 9.4% in low-age patients positive for MMSE items (both P < 0.05). Compared with patients negative for MoCA items, living ability increased by 3.5% in low-age patients positive for MoCA items ( P < 0.05). Correlation analysis revealed that total scores of MMSE and MoCA were significantly negatively correlated with ADL score ( r = -0.26, -0.27, both P < 0.001) and instrumental activity of daily living score ( r = -0.27, -0.27, P < 0.001). Conclusion:Cognitive function and living ability are correlated in older adult patients living in a mining community. We should pay attention to the screening results of cognitive disorder in older adult patients and improve their living ability by improving their cognitive function.

Cognitive function changes and their influential factors in patients with ischemic stroke and leukoaraiosis / 中国基层医药

Objective:To investigate cognitive function changes and their influential factors in patients with ischemic stroke and leukoaraiosis.Methods:A total of 500 patients with ischemic stroke who received treatment in Yiwu Central Hospital from January 2018 to October 2019 were included in this study. They were divided into simple ischemic stroke group ( n = 200) and ischemic stroke complicated by leukoaraiosis group (combination group, n = 300). The infarct location and the degree of leukoaraiosis in the combination group were analyzed. An additional 150 volunteers who concurrently underwent the Cognitive Function Test in the same hospital were selected as controls. Cognitive function was evaluated using the Mini-Mental State Examination (MMSE) and the Montreal Cognitive Assessment (MoCA). Patients in the combination group were divided into cognitive impairment group (MoCA score ≥ 26 points) and non-cognitive impairment group (MoCA score < 26 points) according to MoCA score. The risk factors of cognitive impairment in patients with ischemic stroke and leukoaraiosis were analyzed. Results:The scores of the MMSE, MoCA, Clock Drawing Test (CDT), Verbal Fluency Test (VFT), and Digit Span Test (DST) in the control group were (28.93 ± 2.70) points, (28.35 ± 2.74) points, (4.69 ± 1.14) points, (4.94 ± 0.42) points, and (14.33 ± 1.66) points respectively. They were (26.92 ± 2.18) points, (25.02 ± 3.52) points, (3.61 ± 1.60) points, (4.77 ± 0.46) points, and (11.73 ± 1.16) points, respectively in the simple ischemic stroke group and (24.91 ± 2.79) points, (20.70 ± 3.06) points, (2.87 ± 1.23) points, (4.07 ± 0.85) points, and (10.82 ± 0.93) points respectively in the combination group. There were significant differences in the scores of the MMSE, MoCA, CDT, VFT, and DST among the three groups ( F = 124.50, 318.50, 93.43, 112.60, 428.60, all P < 0.001). Significant differences in the scores of the MMSE, MoCA, CDT, VFT, and DST were observed between patients with different degrees of leukoaraiosis ( F = 69.09, 102.40, 20.98, 60.90, 57.00, all P < 0.001). Spearman correlation analysis results showed that the scores of the MMSE, MoCA, CDT, VFT, and DST were negatively correlated with the degree of leukoaraiosis ( r = -0.61, -0.69, -0.43, -0.56, -0.44, all P < 0.05). Logistic regression analysis results showed that age, history of smoking and drinking, history of diabetes, history of stroke, and infarct location were the independent risk factors for cognitive impairment in patients with ischemic stroke and leukoaraiosis. Education level was a protective factor against ischemic stroke and leukoaraiosis. Conclusion:The degree of cognitive impairment in patients with ischemic stroke and leukoaraiosis is related to the degree of leukoaraiosis. Age, history of smoking and drinking, history of diabetes, history of stroke, infarction location, and education level are the influential factors of cognitive impairment.

Clinical correlates of social cognition after an ischemic stroke: preliminary findings / Correlatos clínicos da cognição social após acidente vascular cerebral isquêmico: descobertas preliminares

ABSTRACT. The co-occurrence of post-stroke behavioral disorders and cognitive impairment has been extensively investigated. However, studies usually do not include social cognition among the assessed cognitive domains. Objective: To investigate the potential association between facial emotion recognition, a measure of social cognition, and behavioral and cognitive symptoms in the subacute phase of ischemic stroke. Methods: Patients admitted to a Stroke Unit with ischemic stroke were followed up to 60 days. At this time point, they were evaluated with the following tools: Mini-Mental State Examination (MMSE); Frontal Assessment Battery (FAB); Visual Memory Test of the Brief Cognitive Battery (VMT); Phonemic Verbal Fluency (F-A-S Test); Digit Span; Facial Emotion Recognition Test (FERT) and Hospital Anxiety and Depression Scale (HADS). A control group composed of 21 healthy individuals also underwent the same evaluation. Results: Eighteen patients with ischemic stroke were enrolled in this study. They had similar age, sex and schooling years compared to controls. Depression symptoms and episodic memory deficits were significantly more frequent in patients compared to controls. The recognition of sadness expression positively correlated with the levels of anxiety and depression, while and the recognition of fear expression negatively correlated with depression in the stroke group. Conclusions: After an ischemic stroke, patients exhibit impairment in social cognition skills, specifically facial emotion recognition, in association with behavioral symptoms.

RESUMO. A co-ocorrência de distúrbios comportamentais e comprometimento cognitivo pós-acidente vascular cerebral (AVC) é amplamente descrita na literatura. No entanto, os estudos geralmente não incluem a cognição social entre os domínios cognitivos avaliados. Objetivo: Investigar a potencial associação entre o reconhecimento da emoção facial, uma medida da cognição social, e os sintomas comportamentais e cognitivos na fase subaguda do AVC isquêmico. Métodos: Pacientes internados em uma Unidade de AVC com AVC isquêmico foram acompanhados até 60 dias, quando foram avaliados com os seguintes instrumentos: Mini-Exame do Estado Mental (MEEM); Bateria de Avaliação Frontal (FAB); Teste de Memória Visual da Bateria Cognitiva Breve (VMT); Fluência Verbal Fonêmica (Teste F-A-S); Span de dígitos; Teste de Reconhecimento de Emoção Facial (FERT) e Escala Hospitalar de Ansiedade e Depressão (HADS). Um grupo controle constituído por 21 indivíduos saudáveis também foi submetido à mesma avaliação. Resultados: Dezoito pacientes com AVC isquêmico foram incluídos no presente estudo, apresentando idade, sexo e anos de escolaridade semelhantes aos do grupo controle. Os sintomas de depressão e déficits de memória episódica foram significativamente mais frequentes em pacientes com AVC. O reconhecimento da expressão de tristeza correlacionou-se positivamente com os níveis de ansiedade e depressão, ao passo que o reconhecimento da expressão de medo correlacionou-se negativamente com depressão no grupo de AVC. Conclusões: Após um AVC isquêmico, pacientes podem apresentar alterações de cognição social, especificamente de reconhecimento da emoção facial, em associação com sintomas comportamentais.

Progress on association of perinatal depression and neurobehavioral development in offspring / 中华围产医学杂志

Perinatal depression can cause adverse effects not only on maternal physical and mental health, but also abnormalities or damage to the brain structure and function of the offspring, resulting in short- or long-term effects on neurobehavioral development. This review summarizes the progress in this field and elaborates the mechanism from a neuroendocrinology and epigenetics point of view.

Efficacy and safety of hydrochloridememantine or donepezil combined with risperidone in treatment of Alzheimer′s disease patients with behavioral and psychological symptoms / 中华全科医师杂志

One hundred Alzheimer′s disease (AD) patients with behavioral and psychological symptoms of dementia (BPSD) treated with donepezil+risperidone group ( n=50, group 1) or hydrochloride memantine+risperidone group ( n=50, group 2) in geriatric departments of Hangzhou Seventh People′s Hospital were enrolled in the study. Montreal cognitive assessment scale (MoCA), positive and negative syndrome scale (PANSS), treatment emergent symptom scale (TESS) were applied for evaluation; and blood routine examination, blood biochemistry, eletrocardiogram were performed in two groups before and after treatment. After treatment, MoCA score in group 2 (16.10±3.90) was significantly higher than that in group 1 (18.14±3.71)( t=-3.99, P<0.01), and PANSS score in group 2 (86.66±6.62) was significantly lower than that in group 1 (109.50±11.51; t=12.67, P<0.01). The incidence rates of dry mouth (16%,8/50), extrapyramidal side effects (10%,5/50) and the total score of TESS (3.92±2.02) in group 2 were markedly lower than those of group 1 [44%(22/50), 36%(18/50), (12.49±1.45);χ 2=9.33,9.54, t=17.90,all P<0.01]. Meanwhile, group 2 had significantly lower influences on the levels of blood lipids and fasting blood glucose (all P<0.01). The risperidone dose used in group 2 was significantly lower than that in group 1 [(2.06±0.50) vs. (3.85±0.89)mg, t=14.40, P=0.04].The results suggest that hydrochloridememantine combined with risperidone is more effective with less side effects compared to donepezil combined with risperidone for AD patients with BPSD.

Procesos cognitivos en trombosis de seno longitudinal superior: reporte de caso / Cognitive processes in superior longitudinal sinus thrombosis: case report

INTRODUCCIÓN: la trombosis de seno longitudinal superior es una enfermedad de difícil detección a causa de sus diferentes causas y debido al polimorfismo de sus manifestaciones neurológicas; además es común en el sexo femenino relacionado a los estímulos estrogénicos y otros factores hormonales, pero poco probable en menor de 40 años. PACIENTE: paciente menor de 40 años posterior a presentar dos episodios de accidentes cerebrovasculares isquémicos, se evalúa un año después de la lesión el estado cognitivo y funcional mediante la batería Neuropsi Atención y Memoria y el Inventario de Adaptabilidad Mayo-Portland; los procesos cognitivos afectados en un nivel de clasificación leve fueron la atención y concentración, el funcionamiento ejecutivo; en el estado funcional reportado por el cónyuge sugiere afectación de síntomas clínicos como irritabilidad, ira agresividad, dolores de cabeza, cansancio y reacciones a síntomas menores e interacción inadecuada, estos síntomas no fueron reportados por la paciente sugiriendo posible anosognosia en su proceso de adaptabilidad CONCLUSIÓN: Tras un proceso de lesión cerebral por afectación trombótica, las secuelas cognitivos y funcionales permanecen posterior a la recuperación espontanea, llevando a emplear procesos de rehabilitación.

INTRODUCTION: the superior longitudinal sinus thrombosis is a disease difficult to detect because of its different causes and because of the polymorphism of its neurological manifestations; It is also common in females related to estrogen stimuli and other hormonal factors, but unlikely in younger than 40 years. PATIENT: a patient younger than 40 years after having two episodes of ischemic strokes, cognitive and functional status is assessed one year after the injury using the neuropsi battery attention and memory and the mayo-portland adaptability inventory; Cognitive processes affected at a slight level of classification were attention and concentration, executive functioning; In the functional status reported by the spouse suggests involvement of clinical symptoms such as irritability, anger, aggression, headaches, fatigue and reactions to minor symptoms and inadequate interaction, these symptoms were not reported by the patient suggesting possible anosognosia in her process of adaptability CONCLUSIÓN: After a process of brain injury due to thrombotic involvement, the cognitive and functional sequelae remain after spontaneous recovery, suggesting the import of rehabilitation processes.

The Gut Brain-Axis in Neurological Diseases

Abstract Recent evidence suggests that dysfunction of the gut-brain axis may be an important factor contributing to many diseases of the nervous system. Increased gut permeability associated with chronic gastrointestinal dysfunction, as well as changes in the composition of the gut microbiota could contribute to exposure of the enteric and central nervous system to pathogens and its metabolites, including endotoxins and pro-inflammatory cytokines. As a consequence, dysfunction of the host's immune system could contribute to an abnormal immunological response leading to auto-immune conditions, such as multiple sclerosis. So far, gut dysbiosis has been reported in association with Parkinson's disease, Alzheimer's disease, multiple sclerosis, neurodevelopmental and neuropsychiatric conditions, and cerebrovascular disease. These findings suggest that the possibility of targeting the gut microbiota could become a future therapeutic option to treat these conditions. However, before this knowledge can be useful in the clinical setting, more data is needed to establish clear causal relationships between dysfunction of the gut-brain axis and neurological diseases.

Challenges in the diagnosis of dementia: insights from the United Kingdom-Brazil Dementia Workshop / Desafios no diagnóstico de demência: conclusões do Workshop de Demência Reino Unido-Brasil

ABSTRACT. In July 2019, a group of multidisciplinary dementia researchers from Brazil and the United Kingdom (UK) met in the city of Belo Horizonte, Minas Gerais, Brazil, to discuss and propose solutions to current challenges faced in the diagnosis, public perception and care of dementia. Here we summarize the outcomes from the workshop addressing challenges in diagnosis. Brazil faces a major problem in dementia underdiagnosis, particularly involving the population in an adverse socioeconomic context. There is poor availability of resources and specialists, and the knowledge of general practitioners and other healthcare professionals is far from satisfactory. Low education level is a further obstacle in diagnosing dementia, as the most commonly used screening tests are not designed to evaluate this population. Patients and their families must overcome the stigma of a diagnosis of dementia, which is still prevalent in Brazil and increases the burden of this condition. Whilst the UK has greater resources, dedicated memory services and a National Dementia Strategy plan, the National Health Service (NHS) has limited funding. Therefore, some challenges regarding diagnosis are common across both countries. The authors suggest possible solutions to confront these, with the goal of improving assessment and recognition of dementia and reducing misdiagnosis.

RESUMO. Em julho de 2019, um grupo multidisciplinar de pesquisadores em demência do Brasil e do Reino Unido se reuniu em Belo Horizonte para discutir e propor soluções para os desafios no diagnóstico, percepção pública e tratamento dessa condição. Neste artigo, sintetizamos as conclusões do workshop sobre os desafios no diagnóstico de demência. O Brasil enfrenta um grande problema no subdiagnóstico de demência, principalmente entre a população em condições socioeconômicas adversas. Há pouca disponibilidade de recursos e de especialistas e o conhecimento de médicos generalistas e de outros profissionais de saúde é pouco abrangente. Baixa escolaridade é também um obstáculo no diagnóstico de demência, uma vez que os testes de rastreio mais utilizados na prática clínica não são estruturados para avaliar a população com esse perfil. Os pacientes com demência e suas famílias ainda têm que superar o estigma do diagnóstico, que é ainda muito prevalente no Brasil e colabora para a piora da qualidade de vida. O Reino Unido, por outro lado, dispõe de mais recursos financeiros e de pessoal, possui serviços dedicados à avaliação de problemas de memória e um plano estratégico nacional para demência. Contudo, o National Health Service (NHS) tem verbas limitadas, o que faz com que alguns dos desafios no diagnóstico de demência sejam comuns aos dois países. Os autores sugerem possíveis soluções para enfrentá-los, com o objetivo de melhorar a avaliação e o reconhecimento da demência e reduzir os erros de diagnóstico.

You are not yourself when you are hungry: a rare case of malignant insulinoma

We present a rare case of high-grade functional neuroendocrine carcinoma of the pancreas secreting insulin. Our patient, an 80 years old woman, presented with neuropsychiatric symptoms consistent with hypoglycaemia that regressed with food intake and dextrose administration. Abdominal imaging showed a pancreatic tumour with invasion of the spleen and lymph node metastasis, highly suggestive of an insulinoma as the cause of the hypoglycaemia. The patient underwent left pancreatectomy with splenectomy and atypical gastric resection. The postoperative course of our patient was uneventful, with complete remission of the hypoglycaemic episodes, and the definitive histological examination showed three poorly differentiated large cell neuroendocrine carcinomas of the pancreas.

The blood lead level and its relationship with behavior in school-age children from rural areas of Chongqing / 中华预防医学杂志

Objective@#To explore the blood lead level and its relationship with behavior in school-age children from rural areas of Chongqing.@*Methods@#A total of 697 students from grades 3 to 6 in the fall semester of 2014 from 14 rural townships in one district of Chongqing was selected by using the random cluster sampling method. Blood were sampled to analyze the lead level. Neurobehavioral tests were performed to determine their personal cognitive and memory ability. Questionnaires and physical examinations were administered to obtain the information of confounding factors. All students were divided into Q1-Q4 groups according to the quartile of their blood lead level. The relationship between the blood lead level and behavior was analyzed by multivariate logistic regression model and restricted spline regression model.@*Results@#The mean age of 697 students was (10.07±1.36) years old, and the median (interquartile range) of their blood lead level was 44.31 (35.42) μg/L. Multivariate logistic regression model showed that after adjusting for age, gender, body mass index and maternal culture level, compared with Q1 group, the OR (95%CI) values of high digit symbol substitution test (DSST) scores and high overall memory quotient (MQ) scores in Q3 group were 1.65 (1.01-2.70) and 2.10 (1.21-3.62), and the OR (95%CI) value of high long term memory (LTM) scores in Q4 group was 0.53 (0.31-0.92). The results of the restricted spline regression model showed that the dose-response curves between the blood lead level and MQ/LTM test scores were both parabolic (P<0.05).@*Conclusion@#The blood lead level of school-age children from rural areas of Chongqing is the same as that from other areas of China, but slightly higher than that from other areas of Chongqing. Children with higher blood lead level have poor long-term memory ability.

Related factors for early neurological improvement after intravenous thrombolysis with alteplase in elderly patients with acute ischemic stroke / 中华老年医学杂志

Objective@#To analyze related factors for early neurological improvement after intravenous thrombolysis with alteplase in elderly patients with acute ischemic stroke(AIS).@*Methods@#Clinical data of elderly AIS patients with intravenous thrombolytic therapy within 4.5 hours of symptom onset were retrospectively analyzed.The demographic and clonical data were compared between improvement group and non-improvement group.Relevant factors for early neurological improvement after thrombolysis with alteplase were analyzed by using multivariate logistic regression model.@*Results@#A total of 118 patients were included, of which 43 had early symptom improvement(36.44%). Multivariate logistic regression analysis showed that baseline NIHSS score(OR=1.098, 95%CI=1.002-1.202, P=0.045), admission blood glucose(OR=1.377, 95%CI=1.040-1.822, P=0.025), atrial fibrillation(OR=0.329, 95%CI=0.122-0.883, P=0.027)were significantly correlated with early symptom improvement after thrombolysis.@*Conclusions@#Baseline NIHSS score, admission blood glucose and atrial fibrillation are correlated with early neurological improvement after intravenous thrombolysis with alteptase in elderly AIS patients.

Related factors for early neurological improvement after intravenous thrombolysis with alteplase in elderly patients with acute ischemic stroke / 中华老年医学杂志

Objective To analyze related factors for early neurological improvement after intravenous thrombolysis with alteplase in elderly patients with acute ischemic stroke (AIS).Methods Clinical data of elderly AIS patients with intravenous thrombolytic therapy within 4.5 hours of symptom onset were retrospectively analyzed.The demographic and clonical data were compared between improvement group and non-improvement group.Relevant factors for early neurological improvement after thrombolysis with a[teplase were analyzed by using multivariate logistic regression model.Results A total of 118 patients were included,of which 43 had early symptom improvement (36.44％).Multivariate logistic regression analysis showed that baseline NIHSS score(OR =1.098,95％CI=1.002-1.202,P=0.045),admission blood glucose(OR =1.377,95％CI=1.040-1.822,P =0.025),atrial fibrillation(OR =0.329,95 ％ CI =0.122-0.883,P =0.027) were significantly correlated with early symptom improvement after thrombolysis.Conclusions Baseline NIHSS score,admission blood glucose and atrial fibrillation are correlated with early neurological improvement after intravenous thrombolysis with alteptase in elderly AIS patients.

Outcomes of a Physician Survey on the Type, Progression, Assessment, and Treatment of Neurological Disease in Mucopolysaccharidoses

Abstract The mucopolysaccharidosis (MPS) disorders are a group of rare, inherited lysosomal storage disorders. In each of the 11 MPS (sub)types, deficiency in a specific lysosomal enzyme (1 of 11 identified enzymes) leads to accumulation of glycosaminoglycans, resulting in cell, tissue, and multi-organ dysfunction. There is great heterogeneity in the clinical manifestations both between and within each MPS type. Somatic signs and symptoms include short stature, coarse facial features, skeletal and joint abnormalities, cardiorespiratory dysfunction, hepatosplenomegaly, and vision and hearing problems. In addition, patients with MPS I, II, III, and VII can have significant neurological manifestations, including impaired cognitive, language, and speech abilities, behavioral abnormalities, sleep problems, and/or epileptic seizures. Hydrocephalus is a frequent finding in patients with MPS I, II, and VI. Spinal cord compression can develop in almost all MPS disorders. Effective management and development of therapies that target these neurological manifestations warrant a profound understanding of their pathophysiology and progression in the different MPS types and best practices for evaluation and treatment. In order to obtain expert opinion addressing these topics we performed an online survey among an international group of experts with extensive experience in managing and treating MPS disorders. The results of this survey provide important insights into the management of neurological manifestations of MPS in clinical practice and are a valuable addition to current evidence.

The predictive value of continuous aEEG monitoring for neurodevelopmental outcome in infants with hypoxic ischemic encephalopathy receiving hypothermia treatment / 中国新生儿科杂志

Objective To study the predictive value of continuous amplitude-integrated electroencephalogram (aEEG) monitoring for the neurodevelopment outcome in infants with hypoxic-ischemic encephalopathy (HIE) receiving hypothermia treatment.Method From April 2014 to May 2016,neonates admitted to our NICU with HIE receiving hypothermia treatment were continuously monitored using aEEG for 96 h,and assigned into moderately and severely abnormal groups according to aEEG results.The aEEG results before hypothermia treatment,within 24 h,48 h,72 h and 96 h after hypothermia treatment were recorded.The Bayley Scales of Infant Development Ⅱ examination was performed at 6 months of age.The sensitivity,specificity,positive and negative predictive values and Youden's index of aEEG for poor outcome at these timepoints was compared.Result A total of 30 neonates were enrolled.Among them 13 were moderately abnormal and 17 were severely abnormal.The gender,gestational age,birth weight and delivery method between two groups were similar (P ＞ 0.05).The 1 min Apgar score,arterial pH,base excess (BE) were significantly lower in the severely abnormal group (P ＜ 0.05).The neurodevelopment assessment at 6 months of age showed unfavorable outcomes in 16 cases,while the remaining 14 cases had generally good outcomes.The sensitivity and specificity of aEEG before hypothermia treatment for the prediction of poor outcome was 81.3％ and 71.4％ respectively.The sensitivity and negative predictive values of aEEG within 24 and 48 after hypothermia treatment for poor outcome was 100％.The Youden's index of aEEG within 72 h after hypothermia treatment for abnormal outcome was the highest 0.661.Conclusion The aEEG before hypothermia treatment alone is not a reliable indicator of poor outcomes in HIE neonates.The aEEG within 72 h after hypothermia is better.Continuous aEEG monitoring during hypothermia in HIE infants is very important because it provides reliable prediction of outcome.

Comprehensive neuropsychological evaluation of children and adolescents with sickle cell anemia: a hospital-based sample

Abstract Background: Individuals with sickle cell anemia may suffer symptomatic or silent cerebral infarcts leading to neurocognitive complications. This study investigated the cognitive and intellectual performance of children and adolescents with sickle cell anemia. Methods: The socioeconomic status, clinical aspects and behavioral profile of 15 young individuals with sickle cell anemia were evaluated. The Wechsler Intelligence Scale for Children, the Developmental Neuropsychological Assessment Test, and the Child Behavior Checklist were applied. Results: Participants with a history of stroke had lower intelligence quotient (IQ) scores. Alterations were found in attention and executive functioning, language, verbal and visual memory, visuospatial processing and sensorimotor skills. These alterations were found both in the children and adolescents who had had a cerebral infarction and in those who apparently had not. In the majority of cases, there were learning difficulties, a history of repeating school years and a need for specialist educational support. The most common additional diagnoses in accordance with the Diagnostic and Statistical Manual of Mental Disorders IV were depressive disorder, anxiety disorder and somatic disorder, as well as conditions associated with physical and psychosocial repercussions of sickle cell anemia. Conclusion: As sickle cell anemia is considered a progressive cerebral vasculopathy, it is a potential risk factor for neurocognitive and psychosocial development. Therefore, periodic neuropsychological and behavioral evaluations of children and adolescents with sickle cell anemia may represent a useful measure to reduce long-term biopsychosocial repercussions.

Histopathological and Neurobehavioral Characterization in Adult Mice Exposed to Traumatic Brain Injury

PURPOSE: Traumatic brain injury (TBI) is a significant cause of morbidity and mortality worldwide. Severity of the initial insult is one of the most significant factors affecting outcome following TBI. In order to investigate the mechanisms of cellular injury and develop novel therapeutic strategies for TBI, we designed a standardized animal TBI model and evaluated histological and functional outcomes according to the degree of impact severity. METHODS: Male adult C57Bl/6 mice underwent controlled cortical impact (CCI) at varying depths of deflection (1.0-2.0 mm). We performed hematoxylin and eosin staining at 7 days after recovery from TBI. Neurobehavioral characterization after TBI was analyzed by the Barnes maze test, passive avoidance test, open field test, rotarod test, tail suspension test, and light/dark test. RESULTS: We observed a graded injury response according to the degree of deflection depths tested (diameter, 3 mm; velocity, 3 m/s; and duration, 500 ms) compared to sham controls. In the Barnes maze test, the severe TBI (2 mm depth) group showed reduced spatial memory as compared with the sham and mild TBI (1 mm depth) groups at 7 days after TBI. There was a significant difference in the results of the open field test and light/dark test among the three groups. CONCLUSION: Our findings demonstrate that the graded injury responses following TBI resulted in differential histopathological and behavioral outcomes in a mouse experimental CCI model. Thus, a model of CCI with histologic/behavioral outcome analysis may offer a reliable and convenient design for preclinical TBI research involving mice.

Effects of squalene on behavior and proteins of glutamate toxicity pathways in mouse model of depression / 药学学报

To study the effects of squalene on behavior and related proteins of glutamate toxicity pathways in the mice with chronic unpredictable mild stress (CUMS), thirteen different kinds of CUMS were applied to the male BALB/C mice for 35 days to establish the mouse model of CUMS depression. The stress conditions include food deprivation, noise, stroboscopic lighting, hot stress (45℃), brake, exposure to lower temperature (4℃), shake, soiled cage, clamp tail, water deprivation, swimming, electric shock, presence of a foreign object in the home cage. The mice were treated with squalene at 3 doses (80, 40 and 20 mg·kg-1·d-1) through oral administration from the 3rd week continuously. Three weeks later, the impacts were evaluated in the mice with behavioral tests, and malondialdehyde (MDA) and hippocampal glutamate (GLU) contents, the superoxide dismutase (SOD) and glutathione peroxidase (GSH-Px) activity in hippocampus were measured by spectropho-tometry or reversed phase HPLC (RP-HPLC). Western blot was used to examine the expression level of N-methyl-D-aspartate receptor subunits epsilon-2 (NMDAε2), calmodulin kinaseⅡ (CaMKⅡ) and neuronal nitric oxide synthase (NOS1) in hippocampus. Compared with model group, the squalene-treated mice exhibited an increase in body weight, sucrose preference rate and the times of crossing-movement and rearing-movement, shortened the immobility time in the tails suspension test and forced swimming test in the depression mice (PPε2, CaMKⅡ and NOS1 in the hippocampus. In conclusion, squalene shows anti-depressant effect on depressant model in mice, meanwhile the downregulated ROS, related proteins of GLU-NMDAε2-CaMKⅡ-NOS1 signal pathways may be related to the antidepressant effect of squalene.

The modelling of the autism spectrum disorder of Sprague-Dawley rat exposed to Poly-IC in the pregnancy and the preliminary study of neuropathologic changes / 中华实用儿科临床杂志

Objective Through modelling autism spectrum disorder(ASD) in condition of Poly-IC,to analyze the development,nervous pathological changes and behavior of rats in experiments,and to define whether the Sprague-Dawley(SD) rats are suitable for the study of ASD.Methods Ten SD rats were randomly divided in 2 groups with 5 rats in each group.The experimental group rats were injected intraperitoneally with 5 mg/kg Poly-IC at gestational day 12,while the rats in control group were injected intraperitoneally with 9 g/L normal saline at the gestational day 12.The body weight,eye-opening time,swimming performance,and conduct parallel water maze test,social communication ability test of each offspring were recorded,and the developmental state of rats and the existence of autism-like social behavior were evaluated.The alterations in hippocampus morphology of offspring rats were indirectly observed with immunofluorescence double standard dyeing.Results Compared with the control group,the weights of the experimental rats were lighter,opening-eye time was delayed,swimming score was lower,in each measurement point.The differences above all were significant(all P ＜0.05).The near-escape scores of experiment rats in social communication ability test were lower than those of rats in control group.The rats in the experimental group needed more time to get the Morris water maze platform,and the times of rightly run through the Morris water maze decreased significantly,and the differences were significant (all P ＜ 0.05).Pathological results revealed that the mineralocorticoid receptor (MR) average optical density value (0.061 3 ± 0.028 8) and the glucocorticoid receptor (GR) mean optical density value (0.041 9 ± 0.040 3) in hippocampal CA1 area of experiment rats were lower than those of the control group (MR:0.081 3 ±0.053 9;GR:0.061 2 ±0.043 6) (t =10.319,10.241;all P ＜0.05).There was no significant difference between MR/GR ratio(the ratio of MR and GR optical density value in the same vision area) in experimental group and in the control group.Conclusions The Poly-IC during early pregnancy can cause lag behind in motor development,harm the social communication ability,lose memory ability to learn,and develop ASD symptoms of SD rat,and these symptoms may be associated with abnormal expression of MR and GR on the hippo-camp cells surface.AS a consequence,early pregnant SD rats exposed to Poly-IC can be used for the establishment of the model of ASD which can provide a platform for the research of ASD.

Neurobehavior and motor development changes in the 3-day-old rats with ischemic brain injury / 中国新生儿科杂志

Object To investigate the pathological changes,physiological condition,neurobehavior and motor development of the 3-day-old rats with ischemic brain injury.Methods Ninety six 3-day-old male Sprague-Dawley rats were randomly assigned to experimental and control groups.Occlusion of both carotid arteries was performed in the experimental group,while the rats in the control group only received skin incisions without carotid ligation.Physical examinations and neurobehavioral development of the rats were recorded daily from the first day after operation until 3 weeks after birth,including weight,eye opening, incisor eruption,ear unfolding,righting reflex,negative geotaxis,limb placing and grasping reflex. Specimens of the brain tissue were obtained in 24 hours after operation and 3 weeks after birth for the hematoxylin-eosin staining and immunohistochemistry staining to investigate the pathological changes.All the reaults were compared between the 2 groups.Results Compared with the control group,rats in the experimental group were found growth retardation,suppression of primitive reflexes and impaired motor abilities (P < 0.05 ).The brain tissue obtained from the rats after operation showed white matter rarefaction,liquefaction and microglia hyperplasia with Hematoxylin-eosin staining and myelin formation disorder with immunohistochemistry staining.Conclusions Ischemic brain injury of the 3-day-old rats could result the mental retardation,neurobehavioral and motor development disorder because of the white matter injury.