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.

Sundown syndrome in patients with Alzheimer's disease dementia / Síndrome do entardecer em pacientes portadores de demência da doença de alzheimer

ABSTRACT The sundown syndrome is a complex neurobehavioral disorder in patients with dementia associated with high financial cost and significant caregiver burden. It is a multifactorial phenomenon with unclear pathophysiology, characterized by the presence of neuropsychiatric symptoms in the evening period. Objective: To analyze the main neuropsychiatric symptoms, their correlation with one another, with comorbidities, and with time of day of greatest symptom intensity in patients with Alzheimer's disease dementia. Methods: This is a cross-sectional, observational and explanatory study in which caregivers/relatives of elderly patients with dementia were interviewed using a structured tool called the Neuropsychiatric Inventory (NPI). Results: The sample studied was composed of 38 patients, 60.5% female and 39.5% male, with mean age of 81±6 (67-94) years. A high frequency of neuropsychiatric symptoms in the evening period was observed, predominantly irritability (55.3%), nocturnal behavior (47.4%), and aggressiveness (42.1%). Only 36.8% of the family caregivers used non-pharmacological strategies. Conclusion: The frequency of neuropsychiatric symptoms was exacerbated in the evening among patients with Alzheimer's disease, especially for those behavioral symptoms that had a positive correlation with one another.

RESUMO A síndrome do entardecer ou "pôr do sol" - "sundown syndrome" - é uma alteração neurocomportamental complexa em pacientes portadores de demência que geram custos financeiros excessivos e desgaste importante a seus cuidadores. Trata-se de um fenômeno multifatorial com fisiopatologia incerta, que caracteriza-se pela presença de sintomas neuropsiquiátricos no período vespertino. Objetivo: Analisar os principais sintomas neuropsiquiátricos, sua correlação entre si, com comorbidades e o horário do dia de maior intensidade destes em pacientes portadores de demência da doença de Alzheimer. Métodos: Trata-se de um estudo analítico transversal, observacional e explanatório no qual foram entrevistados cuidadores/familiares de pacientes idosos portadores de demência utilizando uma ferramenta estruturada denominada Neuropsychiatric Inventory (NPI). Resultados: A amostra estudada foi de 38 pacientes, 60,5% feminino e 39,5% masculino, com 81±6 (67-94) anos de idade. Foi observada uma alta frequência de sintomas neuropsiquiátricos no período vespertino, principalmente irritabilidade (55,3%), comportamento noturno (47,4%), agressividade (42,1%). Apenas 36,8% dos cuidadores familiares empregavam estratégias não farmacológicas. Conclusão: A frequência dos sintomas neuropsiquiátricos são exacerbados ao entardecer em pacientes com doença de Alzheimer; principalmente os sintomas comportamentais com correlação positiva entre si.

Neuropsychiatric Symptoms in Patients with Mild Cognitive Impairment and Dementia of Alzheimer's Type / 정신신체의학

OBJECTIVES: We investigated the prevalence and composite score of the neuropsychiatric symptoms in patients with mild cognitive impairment(MCI), and dementia of Alzheimer's type(AD). The aim of this study is to analyze the correlation between the result of Korean Neuropsychiatric Inventory(K-NPI) and cognitive function. METHODS: A total of 163 patients diagnosed with MCI or AD was divided into three groups(55 MCI patients, 56 dementia patients with mild stage, and 52 dementia patients with moderate, severe stage). We examined neuropsychiatric symptoms by K-NPI and compared the prevalence and composite score of each subdomain in K-NPI among three groups. RESULTS: The most common symptoms in the MCI group were depression/dysphoria, sleep/night-time behavior, anxiety, and irritability/lability. In mild AD group, the most frequent disturbance was agitation/aggression, depression/dysphoria, anxiety, apathy/indifference, and sleep/night-time behavior. In moderate to severe AD group, the most frequent disturbance was apathy/indifference, depression/dysphoria, agitation/aggression, and delusion. The frequencies of delusion, hallucination, agitation/aggression, apathy/indifference, aberrant motor behavior, appetite/eating change were statistically significant. The total NPI score showed a negative correlation with MMSE-KC and a positive correlation with GDS. CONCLUSIONS: Neuropsychiatric symptoms are common features of MCI and AD. These symptoms observed in MCI are similar to those of mild AD. Psychosis is most common in moderate to severe AD, leading to a faster rate of cognitive decline. Therefore, proper management according to the neuropsychiatric symptoms of MCI and three stages of dementia is needed.

Neuropsychiatric Aspects of Traumatic Brain Injury: Symptoms and Pharmacotherapy / 대한정신약물학회지

Traumatic brain injury (TBI) is well recognized as a disability category with high incidence and prevalence as well as considerable impact on survivors and their families. Neuropsychiatric symptoms such as cognitive deficit, psychosis, depression, mania, anxiety are common after TBI. Treatment of the neuropsychiatric symptoms associated with TBI should result in decreased handicap, improved quality of life, and decreased societal impact. This article discusses the clinical approach to treating patients with neuropsychiatric complications following TBI.

A Case of Idiopathic Basal Ganglia Calcification with Dementia

The case of a 66-year-old woman with coexisting idiopathic basal ganglia calcification(IBGC) and dementia was presented. The calcification was detected in bilateral basal ganglia, dentate nucleus, and thalamus by brain imaging. Serum calcium and phosphorus levels were normal. The underlying diseases of calcification of basal ganglia such as parathyroid dysfunction and other infectious, toxic, or metabolic illness were excluded. The patient had memory impairment and frontal executive dysfunction without aphasia, agnosia, apraxia, and visuospatial impairment in neuropsychological test. It suggested that the cognitive impairment might be due to the dysfunction of frontal-subcortical circuit.

Analysis of Influencing Factors on Female Hairdresser's Neuropsychiatric Symptoms Due to Chronic Organic Solvent Exposure / 대한산업의학회지

OBJECTIVES: This study was designed to examine the influencing factors on female hairdresser's neuropsychiatric symptoms due to chronic organic solvent exposure. METHODS: We collected data from 143 female hairdressers employed in 75 hair salons selected by random sampling from the telephone directory of Gangnung City. Using multiple regression analysis we analyzed the influencing factors(i.e. ventilating fans, aircleaners, work duration, total time of exposure to solvents during hair-dyeing and permanent, etc.) on neuropsychiatric symptoms. RESULTS: The neuropsychiatric symptom scores were significantly positively correlated with the total time of hair-dyeing work and work duration, and significantly negatively correlated with the glove wearing proportion. However, the total time of permanent work, ventilating fans and air-cleaners were not significantly correlated. CONCLUSIONS: The results of this study suggest that for the prevention of neuropsychiatric symptoms due to chronic organic solvent exposure, more attention is required regarding hair-dyeing work than permanent work and to skin absorption rather than respiratory inhalation.

Neurobehavioral Performance Changes of Chronic Exposure to Organic Solvents among Textile Processing Workers / 대한산업의학회지

The study was conducted to predict the risk of chronic exposure by neurobehavioral test and to use it as a basic source of the management for workers exposed to organic solvents. The exposed group was selected from coating department of textile processing industry. The unexposed group was selected from those who visited the clinic to take an annual check-up. The author administered WHO-NCTB (neurobehavioral core test battery). Also, Scandinavian Questionnaire 16 was tested by self-administration to find the correlation between subjective symptoms and NCTB results, and to assess the availability of the Questionnaire in mass screening test concurrently. The final analysed subjects were 78 (27 in exposed group and 51 in unexposed group) workers except who had a neurologic disease or systemic disease affecting the central nervous system. In Questionnaire study, there was no significant difference in total neurotoxic symptom scores between the exposed and the unexposed group. In NCTB, the exposed group had lower mean scores than the unexposed group for all tests. When the effect by age and education were considered, there was a statistically significant difference only in pursuit timing test. This study suggests the availability of NCTB and the Questionnaire for the assessment of neurobehavioral change in workers exposed to organic solvents.

Interrelation of Neuropsychiatric Symptom and Neurobehavioral Test among Workers Exposed to Organic Solvents / 대한산업의학회지

A cross-sectional study of 118 car painters and controls of 113 workers was performed to investigate the interrelation between self-reported neuropsychiatric symptom and neurobehavioral performance using Scandinavian Questionnaire 16, Zung anxiety, depression scale and Swedish performance evaluation system (simple reaction time, symbol digit, digit span, finger tapping speed). The typical symptoms characteristic of painter's syndrome were not found and no significant association was found between solvent exposure and !total score of neuropsychiatric symptom but neurobehavioral performance of simple reaction time, symbol digit and digit span had association with solvent exposure. No significant associations were found between total score of neurotoxic symptom, anxiety scale, depression scale and the neurobehavioral performance. In factor analysis, the factor of questionnaire 16 measuring function of memory demonstrated no significant association with the factor of neurobehavioral test items measuring short term memory.