Alcoholic Korsakoff syndrome

酒精中毒 Korsakoff 综合征
  • 文章类型: Journal Article
    背景:长期大量饮酒可能导致永久性脑损伤,认知障碍,和痴呆症。虽然饮酒和犯罪之间的联系很强,几乎没有关于酒精相关性神经认知障碍Wernicke-Korsakoff综合征(WKS)和酒精相关性痴呆(ARD)患者的犯罪行为的研究.
    方法:研究人群包括1998-2015年在芬兰诊断为WKS(n=1149)或ARD(n=2432)的所有患者。诊断数据,死亡率,犯罪是从芬兰全国登记册中获得的。计算诊断前后4年的犯罪率。犯罪类型,发病率,对疾病和普通人群的死亡率进行了比较。
    结果:在确诊前的4年中,总共有35.6%的WKS患者和23.6%的ARD患者犯罪。最常见的是财产和交通犯罪,其次是暴力犯罪。诊断后犯罪行为的发生率明显下降;在WKS患者中,标准化犯罪率(SCR),观察到的犯罪数量与预期犯罪数量的比率(95%CI),诊断前4年为3.91(3.72-4.10),诊断后4年为2.80(2.61-3.00)。同样,在ARD患者中,SCRs诊断前为2.63(2.51-2.75),诊断后为0.84(0.75-0.92).有和没有犯罪史的人之间的死亡率没有显着差异。
    结论:酒精相关神经认知障碍患者在诊断前经常从事犯罪行为,尤其是多重冒犯。在诊断前后的4年里,犯罪率呈线性下降,诊断后明显减少。
    BACKGROUND: Chronic heavy alcohol use may lead to permanent brain damage, cognitive impairment, and dementia. While the link between alcohol use and crime is strong, virtually no research exists on the criminal behavior of patients with the alcohol-related neurocognitive disorders of Wernicke-Korsakoff syndrome (WKS) and alcohol-related dementia (ARD).
    METHODS: The study population included all persons diagnosed with WKS (n = 1149) or ARD (n = 2432) in Finland in 1998-2015. Data on diagnoses, mortality, and crime were obtained from Finnish nationwide registers. Crime incidences were calculated 4 years before and after diagnosis. Crime types, incidences, and mortality were compared between disorders and with the general population.
    RESULTS: Altogether 35.6% of WKS patients and 23.6% of ARD patients had committed crimes in the 4 years preceding diagnosis, most commonly property and traffic crimes, followed by violent crimes. The incidence of criminal behavior decreased significantly after diagnosis; in WKS patients, the standardized criminality ratio (SCR), the ratio of observed to expected number of crimes (95% CI), was 3.91 (3.72-4.10) in 4 years before and 2.80 (2.61-3.00) in 4 years after diagnosis. Likewise, in ARD patients, the SCRs were 2.63 (2.51-2.75) before and 0.84 (0.75-0.92) after diagnosis. No significant difference emerged in mortality between persons with and without a criminal history.
    CONCLUSIONS: Persons with alcohol-related neurocognitive disorders frequently engage in criminal behavior prior to diagnosis, especially multiple offending. In the 4 years before and after diagnosis, crime rates declined in a linear fashion, with a marked reduction after diagnosis.
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  • 文章类型: Journal Article
    Wernicke-Korsakoff综合征是众所周知的硫胺素缺乏的后果,经常与慢性和过度饮酒的患者有关,但它可以由任何导致硫胺素缺乏的原因产生。该疾病未被诊断,因此必须具有高度的临床怀疑,主要是在没有饮酒作为危险因素的患者中。为此,诊断仍然非常临床,具有高临床变异性的困难。补充测试用于支持诊断并排除可能产生类似症状的其他原因,磁共振成像是最具成本效益的成像测试。治疗是基于硫胺素的给药,应该早点开始,以适当的剂量肠胃外,在所有有相容症状的患者中,而不等待确认诊断。
    Wernicke-Korsakoff syndrome is the best known consequence of thiamine deficiency, frequently associated with patients with chronic and excessive alcohol consumption, but it can be produced by any cause that produces thiamine deficiency. The disease is underdiagnosed so it is essential to have a high clinical suspicion, mainly in patients who do not have alcohol consumption as a risk factor. For this, the diagnosis continues to be eminently clinical, with the difficulty of high clinical variability. Complementary tests are used to support the diagnosis and rule out other causes that can produce similar symptoms, with magnetic resonance imaging being the most cost-effective imaging test. Treatment is based on the administration of thiamine, which should be started early, and parenterally at the appropriate doses, in all patients with compatible symptoms, without waiting to confirm the diagnosis.
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  • 文章类型: Journal Article
    OBJECTIVE: In patients with a history of chronic alcohol abuse, neurocognitive disorders (NCD) are not uncommon. The current study aimed to explore the course of cognitive performance, as measured by the Montreal Cognitive Assessment (MoCA), and everyday cognitive functioning, as measured by the Patient Competency Rating Scale (PCRS), in a large group of patients with alcohol use disorder (AUD) admitted to the Center of Excellence for Korsakov and Alcohol-related Cognitive Impairments.
    METHODS: A multiple time-series design was used, in which the MoCA was administered at three time points of assessment, and the PCRS was completed by both the patient and a clinician at two time points, all during clinical treatment.
    RESULTS: A total of 524 patients were included, 71 of whom were diagnosed with AUD only, 284 with AUD and mild NCD (ARCI) and 169 with AUD, major NCD and fulfilling criteria for Korsakoff\'s syndrome (KS).
    CONCLUSIONS: Cognitive performance improved for all three groups during treatment, sustained abstinence and recovery from AUD. A low memory performance on the MoCA without improvement over time was predictive for KS, while improvement on this domain did not differentiate between AUD and ARCI. Changes in overall cognitive performance and orientation in patients with KS were positively related to changes in everyday cognitive functioning.
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  • 文章类型: Journal Article
    目的:Korsakoff综合征(KS)患者表现为执行功能障碍和神经精神症状。本研究调查了特定的执行子组件(移位,更新,和抑制)预测神经精神症状的变化。我们假设不断变化的赤字,特别是,与神经精神症状有关.方法:47名患者参加(平均年龄61.5;11名妇女)。执行功能(EF)使用六个特定于组件的任务进行测量。神经精神症状用神经精神量表-问卷(NPI-Q)测量。使用蒙特利尔认知评估(MoCA)评估一般认知功能。首先,进行因子分析以检查EF任务之间的共享差异。随后,以EF因子和MoCA为预测因子,以NPI-Q为因变量进行回归分析.还研究了EF因子与MoCA之间是否存在相互作用效应。结果:神经精神症状的患病率很高(85.7%的KS患者表现出至少一种症状)。提取了具有移位特定因子和组合更新/抑制因子的双因子模型。总体回归模型不显著,在EF因子和一般认知功能之间没有发现相互作用。然而,检测到一般认知功能和神经精神症状之间存在显著关系(r=-.43;p<.01)。结论:结果表明神经精神症状与一般认知功能之间存在关联。可能,这些严重认知功能障碍患者的认知分化减弱是EF与神经精神症状之间不存在显著关联的原因.虽然由于样本量有限,应谨慎解释结果,发现的关联凸显了进一步阐明KS患者神经精神症状的潜在认知机制的必要性.
    Objective: Patients with Korsakoff\'s syndrome (KS) show executive dysfunction and neuropsychiatric symptoms. This study investigates whether specific executive subcomponents (shifting, updating, and inhibition) predict variance in neuropsychiatric symptoms. We hypothesized that shifting deficits, in particular, are associated with neuropsychiatric symptoms.Method: Forty-seven patients participated (mean age 61.5; 11 women). Executive function (EF) was measured using six component-specific tasks. Neuropsychiatric symptoms were measured with the Neuropsychiatric Inventory - Questionnaire (NPI-Q). General cognitive functioning was assessed with the Montreal Cognitive Assessment (MoCA). First, factor analysis was conducted to examine shared variance across the EF tasks. Subsequently, a regression analysis was performed with the EF factors and the MoCA as predictors and the NPI-Q as the dependent variable. It was also investigated whether an interaction effect between the EF factors and the MoCA was present.Results: The prevalence of neuropsychiatric symptoms was high (85.7% of the KS patients showed at least one symptom). A two-factor model was extracted with a shifting-specific factor and a combined updating/inhibition factor. The overall regression model was not significant, and no interaction was found between the EF factors and general cognitive functioning. However, a significant relationship between general cognitive functioning and neuropsychiatric symptoms (r = -.43; p <.01) was detected.Conclusions: Results point at an association between neuropsychiatric symptoms and general cognitive functioning. Possibly, diminished cognitive differentiation in these patients with severe cognitive dysfunction accounts for the absence of a significant association between EF and neuropsychiatric symptoms. While the results should be interpreted with caution due to a limited sample size, the found association highlights the need to further unravel the underlying cognitive mechanisms of neuropsychiatric symptoms in patients with KS.
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  • 文章类型: Journal Article
    由硫胺素缺乏引起的Wernicke-Korsakoff综合征(WKS)通常被定义为包括脑病,共济失调,和眼肌麻痹。只有16%的尸检证实的WKS患者表现出所有三个迹象。Caine阳性WKS标准包括以下两种或多种:营养缺乏,谵妄或轻度记忆障碍,小脑功能障碍/共济失调,和动眼异常。
    我们描述了精神病住院患者中Caine阳性WKS的患病率,并将治疗前与治疗后的神经认知改善与未受影响组进行了比较。
    这项为期6个月的质量改进评估包括对Caine阳性WKS进行两阶段筛查,给予大剂量静脉内硫胺素(第1天:1200mg;第2-4天:200mg),并在第5天复查。我们使用描述性统计和拟合随机效应模型来检查治疗前/后蒙特利尔认知评估(MoCA)的变化率差异,推迟5项召回,接受治疗的Caine阳性WKS患者和未经治疗的Caine阴性患者之间的步态/协调评分。
    262名患者中,32(12%)患有Caine阳性WKS;17(53%)目前使用酒精。治疗前后Caine阳性WKS(n=26)与Caine阴性比较(n=34)观察到MoCA评分的平均变化(标准偏差)为3.6(2.5)对1.8(2.5)(P<0.01);5项召回:1.8(1.4)对0.5(1.4)(P<0.001);步态/协调评分:-0.6(1.2)对-0.1(0.6)(P<0.001)。动眼异常很少见(Caine阳性WKS中n=4,在Caine阴性比较组中n=2)。
    精神病住院患者中Caine阳性WKS的患病率为12%;只有一半使用酒精。用大剂量硫胺素治疗的患者表现出临床上显着的神经认知改善。
    Wernicke-Korsakoff Syndrome (WKS) resulting from thiamine deficiency is classically defined as including encephalopathy, ataxia, and ophthalmoplegia. Only 16% of autopsy-confirmed patients with WKS exhibit all three signs. Caine-positive WKS criteria include two or more of the following: nutritional deficiency, delirium or mild memory impairment, cerebellar dysfunction/ataxia, and oculomotor abnormalities.
    We describe Caine-positive WKS prevalence among psychiatric inpatients and compare pretreatment-versus-posttreatment neurocognitive improvement to an unaffected group.
    This 6-month quality-improvement evaluation included two-stage screening for Caine-positive WKS, administering high-dose intravenous thiamine (day 1: 1200 mg; days 2-4: 200 mg) with reexamination on day 5. We used descriptive statistics and fitted random effects models to examine rate-of-change differences in pre-/posttreatment Montreal Cognitive Assessment (MoCA), delayed 5-item recall, and gait/coordination scores between treated Caine-positive patients with WKS and untreated Caine-negative patients.
    Of 262 patients, 32 (12%) had Caine-positive WKS; 17 (53%) used alcohol currently. Treated Caine-positive WKS (n = 26) versus Caine-negative comparison (n = 34) before and after treatment observed a mean change (standard deviation) in the MoCA score of 3.6 (2.5) versus 1.8 (2.5) (P < 0.01); 5-item recall: 1.8 (1.4) versus 0.5 (1.4) (P < 0.001); gait/coordination scores: -0.6 (1.2) versus -0.1 (0.6) (P < 0.001). Oculomotor abnormalities were infrequent (n = 4 in Caine-positive WKS, n = 2 in Caine-negative comparison groups).
    Caine-positive WKS prevalence among psychiatric inpatients was 12%; only half used alcohol. Patients treated with high-dose thiamine demonstrated clinically significant neurocognitive improvement.
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  • 文章类型: Journal Article
    经常大量饮酒与广泛的身体有关,心理和社会问题。所有的医疗保健临床医生都应该能够筛查和检测出患者饮酒的问题,并提供有效的简短干预。当酒精依赖患者入院时,必须评估是否需要药物来防止戒断症状和潜在的震颤谵妄和戒断性癫痫发作。应仔细监测和滴定使用长效苯二氮卓类药物如氯二氮卓的药物辅助戒酒效果,临床医生应该意识到Wernicke-Korsakoff综合征和其他并发症的风险。戒酒通常只是治疗的第一步,与社区酒精服务的有效联系是重要的一步。
    Regular heavy consumption of alcohol is associated with a wide range of physical, psychological and social problems. All health-care clinicians should be able to screen for and detect problematic levels of alcohol consumption in their patients, and deliver an effective brief intervention. When patients with alcohol dependence are admitted to hospital there must be an assessment of whether medication is required to prevent withdrawal symptoms and potential delirium tremens and withdrawal seizures. Medically assisted alcohol withdrawal using a long-acting benzodiazepine such as chlordiazepoxide should be carefully monitored and titrated to effect, and the clinician should be aware of the risk of Wernicke-Korsakoff syndrome and other complications. Abstinence from alcohol is usually only the first step in treatment, and effective linkage to community alcohol services is an important step.
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  • 文章类型: Journal Article
    Vitamin deficiencies may reflect less-than-optimal health in select populations. The aim of this study was to determine whether vitamin D supplementation (VDs) after malnutrition may be adversely related to cancer diagnoses in a selected group of patients with alcoholic Wernicke-Korsakoff syndrome (WKS).
    This was a retrospective cohort study of all patients admitted to Slingedael Korsakoff Center, from 1996 to 2018. The patients were subdivided into three predefined groups depending on differences in VDs: \"early\" supplementation, which started during or before the previous hospital admission, before the transfer to our center; \"late\" supplementation, which started later in our center; and \"no\" VDs received. Data collection involved patients\' ages, sex, body mass index, skin type, baseline serum 25-hydroxyvitamin D concentrations if available, doses of cholecalciferol (vitamin D3) supplementation, other vitamins, sun exposure, malnutrition, alcohol use, smoking, cognitive diagnoses, somatic comorbidity, cancer diagnoses, cause of death, and length of stay in Slingedael. New tumors (dependent variable) may have been diagnosed during VDs (exposed cases) or before the start of VDs, if any (unexposed cases).
    New cancers were diagnosed in 87 of 389 (22.4%) patients after median 3 y of follow-up (interquartile range, 1.1‒5.8 y). In logistic regression analysis, age, smoking, and length of stay in log (y) showed odds ratios of 1.021, 2.74, and 1.68, respectively. The temporal relationship of VDs and cancer diagnosis was significant in VDs that started in the year leading up to the diagnosis (Wilcoxon signed-ranks test of positive ranks corresponding with supplementation and negative ranks corresponding with non-supplementation: Z score 2.54; P = 0.011).
    VDs was time-related to cancer diagnosis in a cohort of patients with alcoholic WKS. The study may suggest the proliferation of cancer as an adverse effect of VDs, particularly in malnourished patients.
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  • 文章类型: Journal Article
    丘脑,由几个细胞核组成的中继器官,在前小脑电路和Papez电路之间共享,两者都特别受酒精使用障碍的影响。已知患有Korsakoff综合征的酗酒者比没有神经系统并发症的酗酒者(无并发症的酗酒者)更严重。虽然丘脑萎缩可能是解释Korsakoff综合征健忘症的关键因素,无并发症的酗酒者和有Korsakoff综合征的酗酒者丘脑核内改变的位点和性质尚不清楚。的确,来自动物和人体模型的文献关于丘脑前核是否不同,或者中耳核受到特别影响,会导致健忘症。62名参与者(20名健康对照,26名无并发症的酗酒者和16名Korsakoff综合征患者)接受了扩散张量成像序列和T1加权MRI。根据与前额叶皮层和小脑CrusesI和II的连接,使用最先进的概率示踪图分割丘脑,用于前脑回路的执行回路,前脑回和小脑叶IV-VI为前脑回路的运动回路,帕兹赛道的海马体。计算了这些分区的连通性和体积。示踪图显示,海马主要与丘脑前核相连,而前额叶皮层主要与中背核相连。连接这些脑区及其各自丘脑核的纤维通路也已得到验证。ANCOVA,以年龄和性别为协变量,连通性测量显示,两组患者的丘脑分节仅与海马相连[F(2,57)=12.1;P<0.0001;η2=0.2964;具有从对照组到无并发症的酗酒者再到Korsakoff综合征的连接数量的分级效应]。萎缩,另一方面,在两个患者组中观察到前额叶分裂,与对照组相比程度相同[F(2,56)=18.7;P<0.0001;η2=0.40]。对于海马分裂,仅在Korsakoff综合征组中发现萎缩[F(2,56)=5.5;P=0.006;η2=0.170,使用Bonferroni进行多重比较校正,P<0.01]。对不等样本量的事后Tukey测试,健康对照组>Korsakoff综合征患者(P=0.0036)。两种不同的机制似乎影响丘脑。在前小脑回路中,中耳核的萎缩可能导致改变,而在帕兹赛道上,前核和海马之间的脱节可能是主要因素。前核收缩可能是Korsakoff综合征患者特有的,因此是其病理生理学的潜在神经成像标记,或更普遍的丘脑健忘症,科尔萨科夫综合征历来被用作模型。
    The thalamus, a relay organ consisting of several nuclei, is shared between the frontocerebellar circuit and the Papez circuit, both particularly affected in alcohol use disorder. Shrinkage of the thalamus is known to be more severe in alcoholics with Korsakoff\'s syndrome than in those without neurological complications (uncomplicated alcoholics). While thalamic atrophy could thus be a key factor explaining amnesia in Korsakoff\'s syndrome, the loci and nature of alterations within the thalamic nuclei in uncomplicated alcoholics and alcoholics with Korsakoff\'s syndrome remains unclear. Indeed, the literature from animal and human models is disparate regarding whether the anterior thalamic nuclei, or the mediodorsal nuclei are particularly affected and would be responsible for amnesia. Sixty-two participants (20 healthy controls, 26 uncomplicated alcoholics and 16 patients with Korsakoff\'s syndrome) underwent a diffusion tensor imaging sequence and T1-weighted MRI. State-of-the-art probabilistic tractography was used to segment the thalamus according to its connections to the prefrontal cortex and cerebellar Cruses I and II for the frontocerebellar circuit\'s executive loop, the precentral gyrus and cerebellar lobes IV-VI for the frontocerebellar circuit\'s motor loop, and hippocampus for the Papez circuit. The connectivity and volumes of these parcellations were calculated. Tractography showed that the hippocampus was principally connected to the anterior thalamic nuclei while the prefrontal cortex was principally connected to the mediodorsal nuclei. The fibre pathways connecting these brain regions and their respective thalamic nuclei have also been validated. ANCOVA, with age and gender as covariates, on connectivity measures showed abnormalities in both patient groups for thalamic parcellations connected to the hippocampus only [F(2,57) = 12.1; P < 0.0001; η2 = 0.2964; with graded effects of the number of connections from controls to uncomplicated alcoholics to Korsakoff\'s syndrome]. Atrophy, on the other hand, was observed for the prefrontal parcellation in both patient groups and to the same extent compared to controls [F(2,56) = 18.7; P < 0.0001; η2 = 0.40]. For the hippocampus parcellation, atrophy was found in the Korsakoff\'s syndrome group only [F(2,56) = 5.5; P = 0.006; η2 = 0.170, corrected for multiple comparisons using Bonferroni, P < 0.01]. Post hoc Tukey\'s test for unequal sample sizes, healthy controls > patients with Korsakoff\'s syndrome (P = 0.0036). Two different mechanisms seem to affect the thalamus. In the frontocerebellar circuit, atrophy of the mediodorsal nuclei may lead to the alterations, whereas in the Papez circuit, disconnection between the anterior nuclei and hippocampus may be the leading factor. Shrinkage of the anterior nuclei could be specific to patients with Korsakoff\'s syndrome, hence a potential neuroimaging marker of its pathophysiology, or more generally of thalamic amnesia for which Korsakoff\'s syndrome has historically been used as a model.
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  • 文章类型: Journal Article
    开发性能有效性测试(PVTs)和症状有效性测试(SVTs)以识别出现虚假或夸大症状的人。尽管这两种测试的关键因素包括对认知障碍的相对不敏感,健忘症的直接影响研究甚少。因此,通过神经心理学评估诊断为Korsakoff健忘症(KA)的20例患者样本和20例健康比较(HC)进行了记忆不良测试(TOMM),病态症状的结构化清单(SIMS),以及新开发的视觉关联测试-扩展(VAT-E)。我们的结果表明,KA患者在TOMM和VAT-E上的得分较低,而SIMS的表现与健康比较相当。根据TOMM和VAT-E,一些KA患者被认为表现不佳,提示在严重健忘症中应用这些工具的局限性。在逻辑回归中,PVTs之间存在很强的相互依赖关系。我们得出的结论是,TOMM和VAT-E对严重的记忆障碍并不完全有效,并且存在严重的假阳性风险。需要完整的神经心理学分析,对于疑似健忘症的患者,应谨慎解释PVTs。
    Performance validity tests (PVTs) and Symptom validity tests (SVTs) are developed to identify people that present false or exaggerated symptoms. Although a key factor of both types of tests includes relative insensitivity to cognitive disorders, the direct effects of amnesia have been poorly studied. Therefore, a sample of 20 patients diagnosed with Korsakoff Amnesia (KA) through neuropsychological assessment and 20 healthy comparisons (HC) were administered the Test of Memory Malingering (TOMM), the Structured Inventory of Malingered Symptomatology (SIMS), and the newly developed Visual Association Test - Extended (VAT-E). Our results show that KA patients scored systematically lower on the TOMM and VAT-E, while performance on the SIMS was comparable with healthy comparisons. Some KA patients were regarded as underperformers based on the TOMM and VAT-E, suggesting limitations in applying these instruments in severe amnesia. There was a strong interdependence of PVTs in logistic regression. We conclude that the TOMM and VAT-E are not fully robust against severe memory disorders and show a serious risk of false positives. Complete neuropsychological profile analysis is needed, and PVTs should be interpreted with caution in patients with suspected amnesia.
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  • 文章类型: Case Reports
    The authors present the case of an inpatient hospitalized at the Veterans Affairs psychiatric unit diagnosed with Wernicke-Korsakoff syndrome to promote awareness of this prevalent yet often underdiagnosed and undertreated condition. Although Veterans present with a unique predisposition for alcohol abuse, it remains problematic in the general population as well. Analysis from 2000 to 2003 reveals alcohol use in the past month in Veterans at 56.6% and 50.8% in comparable non-Veterans. According to the National Survey on Drug Use and Health, it is estimated that of those who are 18 and older, 86.4% have used alcohol, 26.9% have engaged in binge drinking in the past month, 7% engaged in heavy alcohol use in the past month, and 6.2% (15.1 million) carried the diagnosis of alcohol use disorder., The lifetime prevalence of alcohol abuse in the general population is estimated to be between 4.5% and 13.2%. Primary care providers should maintain a high degree of vigilance in evaluating patients for timely diagnosis and prompt treatment of those suspected to have thiamine deficiency. Indeed, Wernicke\'s encephalopathy carries a significant level of morbidity and mortality associated with the syndrome, even in cases when it does not present with all of the classic signs. This article aims to raise the primary and ambulatory care provider\'s ability to recognize the condition, emphasize a low threshold to treat, and highlight current treatment recommendations.
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