What is known about the effects of vitamin D in neuropsychiatric lupus?

Abstract Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect several organs and systems. The central and/or peripheral nervous system can suffer from complications known as neuropsychiatric lupus (NPSLE). Studies have associated the manifestations of SLE or NPSLE with vitamin D deficiency. It has been shown that hypovitaminosis D can lead to cognition deficits and cerebral hypoperfusion in patients with NPSLE. In this review article, we will address the main features related to vitamin D supplementation or serum vitamin D levels with neuropsychiatric manifestations, either in patients or in animal models of NPSLE.

nNOS and Neurological, Neuropsychiatric Disorders: A 20-Year Story / 神经科学通报·英文版

In the central nervous system, nitric oxide (NO), a free gas with multitudinous bioactivities, is mainly produced from the oxidation of L-arginine by neuronal nitric oxide synthase (nNOS). In the past 20 years, the studies in our group and other laboratories have suggested a significant involvement of nNOS in a variety of neurological and neuropsychiatric disorders. In particular, the interactions between the PDZ domain of nNOS and its adaptor proteins, including post-synaptic density 95, the carboxy-terminal PDZ ligand of nNOS, and the serotonin transporter, significantly influence the subcellular localization and functions of nNOS in the brain. The nNOS-mediated protein-protein interactions provide new attractive targets and guide the discovery of therapeutic drugs for neurological and neuropsychiatric disorders. Here, we summarize the work on the roles of nNOS and its association with multiple adaptor proteins on neurological and neuropsychiatric disorders.

Correlation between rosacea and neuropsychiatric diseases / 中华皮肤科杂志

Rosacea is a chronic recurrent inflammatory skin disease that mainly occurs on the face. A large number of studies have confirmed that rosacea is related to a variety of systemic diseases, including neuropsychiatric diseases such as anxiety, depression, migraine, Alzheimer′s disease and Parkinson′s disease. This review summarizes research progress in the correlation between rosacea and neuropsychiatric diseases, as well as the underlying mechanisms.

Microglia involved in restricted and repetitive behaviors / 中华行为医学与脑科学杂志

Restricted and repetitive behaviors is the characteristic clinical manifestation of many neuropsychiatric diseases, which seriously affects the work, study and daily communication of patients, and brings huge mental and economic burden to the family and society.Its causes are complex and its manifestations are diverse.Current studies have shown that microglia is closely related to the occurrence of restricted and repetitive behaviors, and the in-depth study of microglia has become a research hotspot to explore the mechanism of restricted and repetitive behaviors.In recent years, studies have found that restricted and repetitive behaviors of various neuropsychiatric diseases (such as frontotemporal dementia, obsessive-compulsive disorder, autism spectrum disorder) are related to microglia.However, reliable evidence for the exact mechanism of microglia involved in restricted and repetitive behaviors remains lacking.This article reviews the recent research progress of microglia involved in restricted and repetitive behaviors.It is expected to provide a new theoretical basis and therapeutic targeting cell for the development and treatment of neuropsychiatric diseases related to restricted and repetitive behaviors in the future.

Systemic lupus erythematosus associated macrophage activation syndrome with neuropsychiatric symptoms: A report of 2 cases / 北京大学学报(医学版)

Systemic lupus erythematosus (SLE) associated macrophage activation syndrome (MAS) is clinically severe, with a high mortality rate and rare neuropsychiatric symptoms. In the course of diagnosis and treatment, it is necessary to actively determine whether the neuropsychiatric symptoms in patients are caused by neuropsychiatric systemic lupus erythematosus (NPSLE) or macrophage activation syndrome. This paper retrospectively analyzed the clinical data of 2 cases of SLE associated MAS with neuropsychiatric lesions, Case 1: A 30-year-old female had obvious alopecia in 2019, accompanied by emaciation, fatigue and dry mouth. In March 2021, she felt weak legs and fell down, followed by fever and chills without obvious causes. After completing relevant examinations, she was diagnosed with SLE and given symptomatic treatments such as hormones and anti-infection, but the patient still had fever. The relevant examinations showed moderate anemia, elevated ferritin, elevated triglycerides, decreased NK cell activity, and a perforin positivity rate of 4.27%, which led to the diagnosis of "pre-hemophagocytic syndrome (HPS)". In May 2021, the patient showed mental trance and babble, and was diagnosed with "SLE-associated MAS"after completing relevant examinations. After treatment with methylprednisolone, anti-infection and psychotropic drugs, the patient's temperature was normal and mental symptoms improved. Case 2: A 30-year-old female patient developed butterfly erythema on both sides of the nose on her face and several erythema on her neck in June 2019, accompanied by alopecia, oral ulcers, and fever. She was diagnosed with "SLE" after completing relevant examinations, and her condition was relieved after treatment with methylprednisolone and human immunoglobulin. In October 2019, the patient showed apathy, no lethargy, and fever again, accompanied by dizziness and vomiting. The relevant examination indicated moderate anemia, decreased NK cell activity, elevated triglycerides, and elevated ferritin. The patient was considered to be diagnosed with "SLE, NPSLE, and SLE-associated MAS". After treatment with hormones, human immunoglobulin, anti-infection, rituximab (Mabthera), the patient's condition improved and was discharged from the hospital. After discharge, the patient regularly took methylprednisolone tablets (Medrol), and her psychiatric symptoms were still intermittent. In November 2019, she developed symptoms of fever, mania, and delirium, and later turned to an apathetic state, and was given methylprednisolone intravenous drip and olanzapine tablets (Zyprexa) orally. After the mental symptoms improved, she was treated with rituximab (Mabthera). Later, due to repeated infections, she was replaced with Belizumab (Benlysta), and she was recovered from her psychiatric anomalies in March 2021. Through the analysis of clinical symptoms, imaging examination, laboratory examination, treatment course and effect, it is speculated that the neuropsychiatric symptoms of case 1 are more likely to be caused by MAS, and that of case 2 is more likely to be caused by SLE. At present, there is no direct laboratory basis for the identification of the two neuropsychiatric symptoms. The etiology of neuropsychiatric symptoms can be determined by clinical manifestations, imaging manifestations, cerebrospinal fluid detection, and the patient's response to treatment. Early diagnosis is of great significance for guiding clinical treatment, monitoring the condition and judging the prognosis. The good prognosis of the two cases in this paper is closely related to the early diagnosis, treatment and intervention of the disease.

Research and application of model fruit fly in prevention and treatment of neuropsychiatric disorders by traditional Chinese medicine and natural compounds: a review / 中国中药杂志

As an important model animal, fruit fly is characterized by outstanding genetic characteristics, relatively perfect nervous system, rapid reproduction, and low cost. Thus, it has been applied in the research on neuropsychiatric disorders in recent years, showing great potential in life science. The incidence of neuropsychiatric disorders has been on the rise, and the disorders have high disability rate and low case fatality rate. The global drug demand for such diseases is second only to cardiovascular and cerebrovascular diseases. At the moment, the demand of the drugs for the diseases have been rising, and it is an urgent task to develop related drugs. However, the research and development of the drugs are time-intensive and have a high failure rate. A suitable animal model can help shorten the time for drug screening and development, thereby reducing the cost and failure rate. This study reviews the application of fruit flies in several common neuropsychiatric disorders, which is expected to provide new ideas for the research and application of the model animals in traditional Chinese medicine.

Review of traditional Chinese medicines in ameliorating neuropsychiatric diseases by improving the levels of monoamine neurotransmitters via gut microbiota regulation / 中国中药杂志

The degeneration of monoaminergic system and the reduction of monoamine neurotransmitters(MNTs) are associated with the occurrence of a variety of neuropsychiatric diseases, becoming the key indicators for clinical diagnosis and treatment. Recent studies suggested gut microbiota could influence the occurrence, development, and treatment of neuropsychiatric diseases by directly or indirectly regulating the synthesis and metabolism of MNTs. Rich clinical experience has been accumulated in the amelioration and treatment of neuropsychiatric diseases by traditional Chinese medicines. The traditional oral administration method demonstrates obvious advantages in regulating gut microbiota. It provides a new idea for explaining the pharmacodynamic material basis and mechanism of traditional Chinese medicines in ameliorating neuropsychiatric disease by improving the levels of MNTs via gut microbiota regulation. Focusing on three common neuropsychiatric diseases including Alzheimer's disease, Parkinson's disease, and major depression, we summarized the pathways of gut microbiota in regulating the levels of MNTs and the paradigms of traditional Chinese medicines in ameliorating neuropsychiatric diseases via the "bacteria-gut-brain axis", aiming to provide ideas for the development of drugs and treatment schemes.

nNOS and Neurological, Neuropsychiatric Disorders: A 20-Year Story / 神经科学通报·英文版

In the central nervous system, nitric oxide (NO), a free gas with multitudinous bioactivities, is mainly produced from the oxidation of L-arginine by neuronal nitric oxide synthase (nNOS). In the past 20 years, the studies in our group and other laboratories have suggested a significant involvement of nNOS in a variety of neurological and neuropsychiatric disorders. In particular, the interactions between the PDZ domain of nNOS and its adaptor proteins, including post-synaptic density 95, the carboxy-terminal PDZ ligand of nNOS, and the serotonin transporter, significantly influence the subcellular localization and functions of nNOS in the brain. The nNOS-mediated protein-protein interactions provide new attractive targets and guide the discovery of therapeutic drugs for neurological and neuropsychiatric disorders. Here, we summarize the work on the roles of nNOS and its association with multiple adaptor proteins on neurological and neuropsychiatric disorders.

A Comparative Assessment of Comprehensive Trail Making Test and Wisconsin Card Sorting Test Among Alcohol Dependence Patients

@#Introduction: Alcohol, when used frequently, accelerates the ageing process, causes brain damage, and results in a reduced volume of grey and white matter, leading to frontal lobe abnormalities. The neurotoxicity resulting from alcohol overuse affects the higher functions of the brain. This study aimed to evaluate the effect of alcohol dependence on the executive functioning of the brain. Methods: This study was carried out as a case-control study among 60 patients with alcohol dependence and 60 controls. Assessment of executive function was carried out using the Comprehensive trail-making test (CTMT) and the Wisconsin card sorting test (WCST). Comparison between the alcohol dependence group and normal healthy controls were calculated using the Mann-Whitney U test as data followed a non-parametric distribution. Results: The mean age of the participants among the cases and controls was 38.3±5.5 years and 37.8±5.4 years, respectively. The results showed a significant difference in both WCST and CTMT between cases and controls (p<0.05). Conclusion: This study concludes that there was an impaired performance in executive functions in alcohol- dependence patients in early abstinence compared to normal controls showing frontal lobe impairment in alcohol-dependence patients.

PANS-PANDAS, reporte de caso / PANS-PANDAS, Case Report

RESUMEN Introducción: Desde 1980 se conocen casos de síndromes neuropsiquiátricos infantiles en el mundo y su concepto ha evolucionado con cambios en las definiciones de 1995 (PITANDS: trastornos neuropsiquiátricos pediátricos autoinmunes precipitados por infección), 1998 (PANDAS: síndrome neuropsiquiátrico autoinmune pediátrico asociado con la infección por estreptococos), 2010 (PANS: síndrome pediátrico neuropsiquiátrico de inicio agudo) y 2012 (CANS: síndromes neuropsiquiátricos agudos de los niños). A pesar de que se conoce desde hace más de 20 años, aún es una enfermedad que suele pasar inadvertida para muchos profesionales de la salud. Objetivo: Sensibilizar a la comunidad médica acerca de la identificación de la enfermedad y disminuir la morbilidad asociada con un diagnóstico tardío. Caso clínico: Una niña de 6 años consultó a urgencias por trastorno de rechazo alimentario. En el tratamiento hospitalario se identificó historia clínica con criterios diagnósticos de PANS-PANDAS. Mostraba un curso clínico recurrente-remitente, tal y como describe la literatura, con pobre respuesta a los tratamientos de primera línea. Conclusiones: En todo niño en edad escolar que se presente con trastorno obsesivo compulsivo o trastornos alimentarios, con sin otros síntomas, se debe evaluar y descartar su asociación con PANS-CANS.

ABSTRACT Introduction: Since 1980, there have been known cases of childhood neuropsychiatric syndromes in the world and its concept has evolved with changes in the definitions in 1995 (PITANDs - paediatric infection-triggered autoimmune neuropsychiatric disorders), 1998 (PANDAS - paediatric autoimmune neuropsychiatric syndrome associated with streptococci infection), 2010 (PANS - paediatric acute-onset neuropsychiatric syndrome) and 2012 (CANS - childhood acute neuropsychiatric syndrome). Despite being known for more than 20 years, it is still an illness that often goes unnoticed by many health professionals. Objective: To sensitise the medical community about the identification of the disease and reduce the morbidity associated with a late diagnosis. Clinical case: A 6-year-old schoolgirl brought to the emergency department due to her refusal to eat. In the hospital treatment, a clinical history was identified with PANS-PANDAS diagnostic criteria. She exhibited a relapsing-remitting clinical course, as described in the literature, with poor response to first-line treatments. Conclusions: In all school-age child presenting with obsessive compulsive disorder or eating disorders, with other symptoms or not, a possible link to PANS-CANS should be evaluated and ruled out.

Relación entre funciones ejecutivas y trastornos neuropsiquiátricos en pacientes con Alzheimer de diferente grado de severidad / Relationship Between Executive Functions and Neuropsychiatric Disorders in Patients with Alzheimer's Disease of Different Degrees of Severity / Relação entre funções executivas e transtornos neuropsiquiátricos em pacientes com Alzheimer de diferentes estágios de gravidade

El objetivo del estudio fue investigar las funciones ejecutivas (fe) y los trastornos neuropsiquiátricos (tn) en pacientes con enfermedad de Alzheimer (ea), en función del grado de severidad, en comparación con sujetos sin deterioro cognitivo (ssdc). Se estudia-ron 50 pacientes con ea y 60 ssdc en un estudio no experimental-transversal con un muestreo no probabilístico basado en una serie de criterios de inclusión. Se aplicó la batería de evaluación frontal y el inventario neuropsiquiátrico, y los resultados mostraron que existe una relación negativa de intensidad moderada entre las fe y lostn, con independencia del grado de severidad de la ea. Se puede concluir que, en fase leve, la euforia se relaciona tanto con la sensibilidad hacia la interferencia como con el control inhibitorio; en fase moderada, la irritabilidad se relaciona de manera positiva con la sensibilidad hacia la interferencia, y en la fase moderadamente grave, la programación motora se relaciona de manera negativa con la agitación.

The aim of the study was to investigate executive func-tions (ef) and neuropsychiatric disorders (nd) in pa-tients with Alzheimer's disease (ad) according to the degree of severity, compared to subjects without cognitive impairment (swci). Fifty patients with ad and 60 swci were studied in a non-experimental-cross-sectional study with non-probabilistic sampling based on a series of inclusion criteria. The frontal evaluation battery and the neuropsychiatric inventory were applied and the results showed that there is a negative relationship of moderate intensity between ef and nd, regardless of the degree of severity of ad. It can be concluded that, in the mild phase, euphoria is related both to sensitivity towards interference and to inhibitory control; in moderate phase irritability is positively related to sensitivity towards interference; and in the moderately severe phase, motor programming is nega-tively related to agitation.

O objetivo do estudo foi investigar as funções executivas (fe) e os transtornos neuropsiquiátricos (tn) em pacien-tes com doença de Alzheimer (da) de acordo com o estágio de gravidade, em comparação com sujeitos sem comprometimento cognitivo (sscc). Foram avaliados 50 pacientes com da e 60 sscc em um estudo transversal não experimental com amostragem não probabilística baseada em uma série de critérios de inclusão. A bateria de avaliação frontal e o inventário neuropsiquiátrico foram aplicados e os resultados mostraram que existe uma relação negativa de intensidade moderada entre a fe e os tn, independente do estágio de gravidade da da. Pode-se concluir que, na fase leve, a euforia está rela-cionada tanto à sensibilidade à interferência quanto ao controle inibitório; na fase moderada, a irritabilidade está positivamente relacionada à sensibilidade à inter-ferência e; na fase moderadamente grave, a programação motora está negativamente relacionada à agitação.

Las orexinas como biomarcadores para demencia tipo Alzheimer y relación con síntomas neuropsiquiátricos / Orexins as biomarkers for Alzheimer dementia and their relationship with neuropsychiatric symptoms

RESUMEN: Los biomarcadores más estudiados en la demencia tipo Alzheimer (DA) son los niveles elevados de Aβ42 y de proteína Tau en líquido cefalorraquídeo. Dada la complejidad de la sintomatología cognitiva y síntomas neuropsiquiátricos (SNP) de esta patología, algunos estudios recientes proponen sustancias como las orexinas, como blanco terapéutico de DA y SNP. El presente trabajo tiene como objetivo revisar publicaciones científicas recientes que hayan analizado la asociación entre orexinas, SNP y DA en humanos, algunos modelos animales y que hayan evaluado a las orexinas como posibles biomarcadores tanto para investigación como en el área clínica. En esta revisión también se describen los estudios que sugieren a las orexinas como un posible biomarcador en la DA, dada su relación con el Aβ42 y la proteína Tau, y otros estudios que las asocian con presencia de SNP, especialmente alteración del sueño. Se plantea la hipótesis de que la presencia de SNP en DA se asocia con las orexinas, debido a que este sistema influye en el funcionamiento hipotalámico y de forma indirecta en áreas cerebrales que regulan el comportamiento. Sin embargo, aún falta mayor investigación, principalmente de estudios longitudinales para conocer claramente la influencia de las orexinas en los SNP.

ABSTRACT The most studied biomarkers in Alzheimer's dementia (AD) are elevated levels of Aβ42 and Tau protein in cerebrospinal fluid. Given the complexity of the cognitive symptomatology and neuropsychiatric symptoms (NPS) of this pathology, some recent studies propose substances such as orexins as a therapeutic target for AD and NPS. The present work aims to review recent scientific publications that have analyzed the association between orexins, PNS and AD in humans. There are some animal models that have evaluated orexins as possible biomarkers both for research and in the clinical area. This review also describes studies that suggest orexins as possible biomarkers in AD, given their relationship with Aβ42 and Tau protein, and other studies that associate them with the presence of SNPs, especially sleep disturbance. It is hypothesized that the presence of SNPs in AD is associated with orexins, because this system influences hypothalamic functioning and indirectly in brain areas that regulate behavior. However, further research is still lacking, mainly longitudinal studies to clearly know the influence of orexins on SNPs.

Active music therapy in dementia: results from an open-label trial / Musicoterapia ativa na demência: resultados de um estudo aberto

OBJECTIVE: Cognitive, neuropsychiatric and functional deficits are core symptoms of dementia. Non- -pharmacological interventions, such as music therapy, when used in conjunction with pharmacological treatment, have the potential to alleviate these symptoms. The purpose of this preliminary study is to examine the active music therapy on cognition and neuropsychiatric symptoms in the elderly with mild and moderate dementia. METHODS: The initial sample consisted of outpatients with dementia (N = 15) and their family members or caregivers (N = 15). Two dyads did not complete the assessments before intervention and were excluded from the analysis. Thirteen females (N = 13) comprised the final sampled and were diagnosed with Alzheimer's disease (N = 10), vascular dementia (N = 2) and mixed dementia (N = 1), at mild (N = 11) and moderate (N = 2) dementia stage. Participants were enrolled in an open-label trial of active music therapy group, set to take place once weekly for 60 minutes over a period of 12 weeks. RESULTS: Participants experienced a slight improvement on cognition measured with Mini-Mental State Examination (p = 0.41), although without statistical significance and a statistically significant decrease in anxiety (p = 0.042) in post-intervention. There were no significant effects on quality of life and caregiver burden. CONCLUSIONS: Active music therapy is a promising intervention with good acceptance among participants. More studies with larger sample sizes are needed to confirm its effects and efficacy in cognitive and neuropsychiatric symptoms in dementia.

OBJETIVO: Distúrbios cognitivos, comportamentais e funcionais são sintomas nucleares na demência. Intervenções não farmacológicas, como a musicoterapia, quando usadas em conjunto com o tratamento farmacológico, têm o potencial de aliviar esses sintomas. O objetivo deste estudo preliminar é examinar a musicoterapia ativa na cognição e nos sintomas neuropsiquiátricos em idosos com demência leve e moderada. MÉTODOS: A amostra inicial foi composta por pacientes ambulatoriais com demência (N = 15) e seus familiares ou cuidadores (N = 15). Duas duplas não completaram as avaliações antes da intervenção e foram excluídas da análise. Treze mulheres (N = 13) compuseram a amostra final e foram diagnosticadas com doença de Alzheimer (N = 10), demência vascular (N = 2) e demência mista (N = 1), nos estágios leve (N = 11) e moderado (N = 2). Os participantes foram inscritos em um estudo aberto de grupo de musicoterapia ativa, programado para ocorrer uma vez por semana, com duração de 60 minutos, durante o período de 12 semanas. RESULTADOS: Os participantes experimentaram uma discreta melhora cognitiva medida pelo Miniexame do Estado Mental (p = 0.41), embora sem significância estatística, e uma diminuição estatisticamente significativa na ansiedade (p = 0.042) na pós-intervenção. Não houve efeitos significativos na qualidade de vida e sobrecarga do cuidador. CONCLUSÕES: A musicoterapia ativa é uma intervenção promissora, com boa aceitação entre os participantes. Mais estudos com amostras maiores são necessários para confirmar seus efeitos e eficácia em sintomas cognitivos e neuropsiquiátricos na demência.

Cerebrovascular changes in neuro-Behcet's disease and neuropsychiatric lupus by transcranial Doppler ultrasonography and magnetic resonance imaging

ABSTRACT Introduction: Transcranial Doppler ultrasonography (TCD) is a technique that allows measurement of blood flow from the basal intracerebral vessels. It is relatively inexpensive, non-invasive, can be performed at the bedside, and allows monitoring in acute emergency settings and for prolonged periods with a high temporal resolution, making it ideal for studying the haemodynamics within the intracranial arteries in neuro-Behcet's disease (NBD) and neuro-psychiatric lupus (NPSLE). Our aim was to assess the cerebral haemodynamic patterns in patients with NBD and NPSLE using TCD, while brain lesions were examined using magnetic resonance imaging (MRI). Material and methods: Case-control prospective study of 30 neuro-Behcet's disease patients, 25 neuro-psychiatric lupus patients and 26 healthy age-matched volunteers. All patients and healthy controls were examined by TCD. Only the groups of patients underwent cranial magnetic resonance imaging (MRI). Results: Transcranial Doppler (TCD) values for middle cerebral artery (MCA), anterior cerebral artery (ACA), posterior cerebral artery (PCA), vertebral artery (VA) and basilar artery (BA) in NBD, NPSLE and control groups were measured. The results showed that there was a significant decrease in mean blood flow velocities in all the arteries examined in NBD and NPSLE patients. There was also a significant increase in the pulsatile index of PCA, VA and BA between NBD and NPSLE patients. The same results were obtained when comparing NBD versus controls. However, there was no significant difference between the NPSLE patients and the control group. The MRI lesions described were parenchymal lesions in 14 patients (46.7%), and vascular lesions in 4 patients (13.3%). Vascular lesions co-existed with parenchymal lesions (mixed lesion). Parenchymal lesions were in white matter (40%), thalamus (26.7%), brain stem (26.7%) and cerebellum (20%). While, in NPSLE, 23 patients were normal (92%) and only two patients had a vascular lesion (8%). Conclusion: There was a significant decrease in mean blood flow and a significant increase in the pulsatile index among both NBD and NPSLE patients, according to the TCD values.

Systemic lupus erythematosus combined with Aspergillus fumigatus brain abscess in 2 cases / 中华神经科杂志

Systemic lupus erythematosus (SLE) complicated with Aspergillus fumigatus brain abscess is rare and needs to be differentiated from bacterial brain abscess and neuropsychiatric lupus. This article reports 2 cases of surgically diagnosed SLE combined with Aspergillus fumigatus brain abscess. The first patient was a 34-year-old woman. Six months after the diagnosis of SLE, she developed convulsions and unconsciousness. She was diagnosed as neuropsychiatric lupus at the first hospitalization because of negative imaging. After discharge, repeated head magnetic resonance imaging revealed abscess-like signals. The second patient, a 20-year-old male, developed high fever, convulsions, and unconsciousness 3 years after the diagnosis of SLE, and head imaging showed an abscess-like signal. The etiology of the cerebrospinal fluid of the 2 patients was both negative, and the Aspergillus fumigatus brain abscess was diagnosed by pathology through abscess resection or drainage. After treatment with voriconazole, the symptoms were relieved and the lesions were subsided.

Clinical efficacy of liver transplantation for Wilson's disease / 中华器官移植杂志

Objective:To explore the clinical efficacy of liver transplantation for Wilson's disease(WD).Methods:From January 1999 to November 2021, clinical data were retrospectively reviewed for 16 recipients with WD undergoing liver transplantation.There were 9 males and 7 females with an age range of 29.5(14~54)years.They were followed up by telephone, outpatient services and hospitalization.The starting point of follow-up was operation date.And recipient death was an endpoint.Postoperative survival, improvement of neuropsychiatric symptom, changes of corneal K-F ring, altered levels of liver function and serum copper-protein at Month 1 post-operation were observed.The follow-up deadline was November 24, 2021.Results:15 recipients underwent classical orthotopic liver transplantation and the other one recipient underwent living-related liver transplantation.No perioperative deaths occurred.All 16 recipients were followed up for 122(6~260)months.The 1-, 5-, and 10-year survival rates were 93.8%、85.2%and 75.8%, respectively.Among 10 recipients with corneal K-F ring positive with varying degrees after operation and was disappeared in 2 recipients at 7 and 11 months.Among 5 recipients with neuropsychiatric manifestation, 4 recipients showed ameliorative neuropsychic symptoms with varying degrees after operation and 1 recipient died.All the levels of liver function and serum copper-protien of all recipients recovered obviously in 1 month and the 1-, 5-, and 10-year post-operation.Conclusions:Classical orthotopic liver transplantation and living-related liver transplantation not only effectively improves copper metabolism of patient with WD and relieves their severe neurological manifestation, but also improves their life and prolongs survival, which is worthy of clinical promotion.

Research Progress in Antibodies Related to Autoimmune Encephalitis in Neuropsychiatric Systemic Lupus Erythematosus / 罕见病研究

Several anti-neural antibodies are associated with neuropsychiatric systemic lupus erythematosus (NPSLE) including anti-neuronal antibodies and anti-glial cell antibodies. The anti-neuronal antibodies has two types: anti-neuronal surface protein antibodies represented by anti-N-methyl-D-aspartate receptor (NMDAR) antibodies, and anti-neuronal intracellular protein antibodies. In this paper, we review and classify the anti-neural antibodies related to NPSLE.

The Relation between Behavioral, Emotional and Cognitive Apathy and Everyday Executive Dysfunction in Alcoholic Korsakoff's Syndrome

ABSTRACT Background: Apathy is an important neuropsychiatric symptom in alcohol-related cognitive impairment in general, and Korsakoff's syndrome in specific. However, research in patients with Korsakoff's syndrome on the multifaceted nature of apathy is lacking. Objectives: Aim of the current study was to examine behavioral, cognitive and emotional apathy in alcoholic Korsakoff patients, also investigating the association with overall cognitive and executive dysfunction. Methods: We studied 43 patients with Korsakoff's syndrome (mean age 60.9, SD=6.5, range 38-70) using the Apathy Evaluation Scale - Informant Version (AES-I) and also administered the Montreal Cognitive Assessment and the Behavior Rating Inventory of Executive Function - Adult Version (BRIEF-A) as a measure of daily executive problems. Results: In our sample, 76% of the Korsakoff patients were classified as being apathetic. AES-I scores correlated with overall cognitive function and were related to observer-rated daily executive problems. Discussion: Apathy is highly prevalent in Korsakoff patients and related to overall cognitive dysfunction and everyday executive problems. Our results stress the need to further examine underlying mechanisms of apathy in Korsakoff patients and the need for interventions aimed at reducing apathy.

Neuropsiquiatría del síndrome de Susac: a propósito de un caso / Neuropsychiatry of Susac Syndrome: a Case Report

RESUMEN El síndrome de Susac es una entidad clínica poco frecuente, posiblemente mediada por un proceso autoinmune; la tríada clásica se compone de retinopatía, disminución en la agudeza auditiva y síntomas neuropsiquiátricos (encefalopatía). Hay pocos casos descritos con sintomatología neuropsiquiátrica como la sintomatología principal. Presentamos un caso de síndrome de Susac, que corresponde a una mujer de 34 arios, con predominio de sintomatologia neuropsiquiátrica, caracterizada por un síndrome de Klüver-Bucy parcial, un síndrome apático, risa y llanto patológico y alteraciones cognitivas de predominio atencional; dichos síntomas mejoraron cualitativamente con el uso de terapia inmunológica. Este caso revela la importancia de las manifestaciones neuropsiquiátricas como presentación clínica en pacientes con entidades neurológicas.

ABSTRACT Susac syndrome is a rare clinical condition, possibly mediated by an autoimmune process; the classic triad is composed of retinopathy, decreased hearing acuity and neuropsychiatric symptoms (encephalopathy). There are few cases reported with neuropsychiatric symptoms as the main manifestation. We present a case of Susac syndrome in a 34-year-old female with a predominance of neuropsychiatric symptoms, characterised by partial Klüver-Bucy syndrome, apathy syndrome, pathological laughter and crying, and cognitive dysfunction predominantly affecting attention, which showed a qualitative improvement with the use of immunological therapy. This case report highlights the importance of neuropsychiatric manifestations as clinical presentation in patients with neurological conditions.

The role of the microbiota-gut-brain axis in neuropsychiatric disorders

The microbiota-gut-brain axis is a bidirectional signaling mechanism between the gastrointestinal tract and the central nervous system. The complexity of the intestinal ecosystem is extraordinary; it comprises more than 100 trillion microbial cells that inhabit the small and large intestine, and this interaction between microbiota and intestinal epithelium can cause physiological changes in the brain and influence mood and behavior. Currently, there has been an emphasis on how such interactions affect mental health. Evidence indicates that intestinal microbiota are involved in neurological and psychiatric disorders. This review covers evidence for the influence of gut microbiota on the brain and behavior in Alzheimer disease, dementia, anxiety, autism spectrum disorder, bipolar disorder, major depressive disorder, Parkinson's disease, and schizophrenia. The primary focus is on the pathways involved in intestinal metabolites of microbial origin, including short-chain fatty acids, tryptophan metabolites, and bacterial components that can activate the host's immune system. We also list clinical evidence regarding prebiotics, probiotics, and fecal microbiota transplantation as adjuvant therapies for neuropsychiatric disorders.