scholarly journals Cognitive Impairment In Stable Wilson Disease Across Phenotype.

Author(s):  
Frederik Teicher Kirk ◽  
Ditte Emilie Munk ◽  
Tea Lund Laursen ◽  
Hendrik Vilstrup ◽  
Peter Ott ◽  
...  

Abstract Background: In Wilson disease (WD), mutations in the gene encoding the ATP7B copper transport protein causes accumulation of copper especially in liver and brain. WD typically presents with hepatic and/or neuropsychiatric symptoms. Impaired cognition is a well-described feature in patients neurological WD, while the reports on cognition in hepatic WD patients are fewer and less conclusive. We examined cognition in a cohort of WD patients with both phenotypes. Methods: In this cross-sectional pilot study, we investigated cognition in 28 stable Danish WD patients by portosystemic encephalopathy (PSE) and continuous reaction time (CRT) tests. Half of the patients were female and median age was 35.5 years (IQR 24.5). The phenotype was hepatic in 14 (50%), neurologic in 10 (36%) and mixed in 4 4 (14%). The duration of treatment was >2 year in all patients, and the condition stable as judged by urinary copper excretion, liver enzymes, and clinical assessment.Results: In total, 16 (57%) patients performed worse than normal in the PSE and/or CRT tests. The two tests correlated (rho=0.60, p=0.0007) with each other, but neither correlated with phenotype, MELD-, Child-Pugh score, 24h-U-Cu, or treatment type.Conclusion: Measurable cognitive impairment was present in more than half of the stable WD patients independent of phenotype. Thus, our data questions the existence of a purely hepatic phenotype.

2017 ◽  
Vol 30 (1) ◽  
pp. 103-113 ◽  
Author(s):  
N. Siafarikas ◽  
G. Selbaek ◽  
T. Fladby ◽  
J. Šaltytė Benth ◽  
E. Auning ◽  
...  

ABSTRACTBackground:Neuropsychiatric symptoms (NPS), such as depression, apathy, agitation, and psychotic symptoms are common in mild cognitive impairment (MCI) and dementia in Alzheimer's disease (AD). Subgroups of NPS have been reported. Yet the relationship of NPS and their subgroups to different stages of cognitive impairment is unclear. Most previous studies are based on small sample sizes and show conflicting results. We sought to examine the frequency of NPS and their subgroups in MCI and different stages of dementia in AD.Methods:This was a cross-sectional study using data from a Norwegian national registry of memory clinics. From a total sample of 4,571 patients, we included those with MCI or AD (MCI 817, mild AD 883, moderate–severe AD 441). To compare variables across groups ANOVA or χ2-test was applied. We used factor analysis of Neuropsychiatric Inventory Questionnaire (NPI-Q) items to identify subgroups of NPS.Results:The frequency of any NPS was 87.2% (AD 91.2%, MCI 79.5%; p < 0.001) and increased with increasing severity of cognitive decline. The most frequent NPS in MCI was depression. Apathy was the most frequent NPS in AD across different stages of severity. The factor analysis identified three subgroups in MCI and mild AD, and a fourth one in moderate–severe AD. We labelled the subgroups “depression,” “agitation,” “psychosis,” and “elation.”Conclusions:The frequency of NPS is high in MCI and AD and increases with the severity of cognitive decline. The subgroups of NPS were relatively consistent from MCI to moderate-severe AD. The subgroup elation appeared only in moderate-severe AD.


2004 ◽  
Vol 16 (1) ◽  
pp. 51-60 ◽  
Author(s):  
Yonas E. Geda ◽  
Glenn E. Smith ◽  
David S. Knopman ◽  
Bradley F. Boeve ◽  
Eric G. Tangalos ◽  
...  

Background: There is inadequate information regarding the neuropsychiatric aspect of Mild Cognitive Impairment (MCI).Objective: To determine the neuropsychiatric profile of MCI, and compare this with normal controls and patients with mild Alzheimer's Disease (AD).Design: Cross-sectional assessment of psychiatric symptoms in subjects that are enrolled in Mayo Clinic's longitudinal study of normal aging, MCI and dementia.Methods and Participants: The Neuropsychiatric Inventory (NPI) was administered to normal control subjects, MCI subjects and patients with early AD. Individual NPI domain scores and total NPI scores were compared among the three groups after controlling for age, educational status, Dementia Rating Scale (DRS) and Mini-Mental State Examination (MMSE) scores. Statistical analysis was performed by utilizing ANOVA, χ2 and Fisher's exact test.Results: Data were analyzed on 514 normal controls, 54 MCI subjects, and 87 subjects with mild AD (CDR of 0.5 or 1); females consisted of 60.3%, 53.7% and 57.5%; and, the average ages (SD) were 77.8 (1.95), 79 (4.6), 80.5 (14.6) respectively. ANOVA pair-wise comparison revealed that both MMSE and DRS differences among the three groups were significantly different at (p=0.05). The total NPI scores were significantly different (p=0.0001, F=107.93) among the three groups using ANOVA. Pair-wise comparison of individual behavioral domain of NPI showed statistically significant differences between MCI and normals; and MCI and AD (p=0.001). Group differences on NPI remained after controlling for age and education at p=0.0375 and p=0.0050 respectively.Conclusion: The neuropsychiatric pattern is reminiscent of the clinical, neuroimaging and neuropsychological profile of MCI. It gives further credence to the view that MCI is indeed the gray zone, with overlap on both ends of the pole.


2013 ◽  
Vol 25 (12) ◽  
pp. 2047-2056 ◽  
Author(s):  
Quincy M. Samus ◽  
Amrita Vavilikolanu ◽  
Lawrence Mayer ◽  
Matthew McNabney ◽  
Jason Brandt ◽  
...  

ABSTRACTBackground:There is a lack of empirical evidence about the impact of regulations on dementia care quality in assisted living (AL). We examined cohort differences in dementia recognition and treatment indicators between two cohorts of AL residents with dementia, evaluated prior to and following a dementia-related policy modification to more adequately assess memory and behavioral problems.Methods:Cross-sectional comparison of two AL resident cohorts was done (Cohort 1 [evaluated 2001–2003] and Cohort 2 [evaluated 2004–2006]) from the Maryland Assisted Living studies. Initial in-person evaluations of residents with dementia (n = 248) were performed from a random sample of 28 AL facilities in Maryland (physician examination, clinical characteristics, and staff and family recognition of dementia included). Adequacy of dementia workup and treatment was rated by an expert consensus panel.Results:Staff recognition of dementia was better in Cohort 1 than in Cohort 2 (77% vs. 63%, p = 0.011), with no significant differences in family recognition (86% vs. 85%, p = 0.680), or complete treatment ratings (52% vs. 64%, p = 0.060). In adjusted logistic regression, cognitive impairment and neuropsychiatric symptoms correlated with staff recognition; and cognitive impairment correlated with family recognition. Increased age and cognitive impairment reduced odds of having a complete dementia workup. Odds of having complete dementia treatment was reduced by age and having more depressive symptoms. Cohort was not predictive of dementia recognition or treatment indicators in adjusted models.Conclusions:We noted few cohort differences in dementia care indicators after accounting for covariates, and concluded that rates of dementia recognition and treatment did not appear to change much organically following the policy modifications.


2021 ◽  
Author(s):  
Pim B. van der Meer ◽  
Johan A.F. Koekkoek ◽  
Martin J. van den Bent ◽  
Linda Dirven ◽  
Martin J.B. Taphoorn

Abstract IntroductionAEDs have been associated with depression, anxiety, and cognitive impairment, all frequent complications of glioma and its subsequent treatment, with considerable morbidity and an adverse effect on health-related quality of life. This study aimed to determine the independent association between AED use and self-reported depression, anxiety, and subjective cognitive impairment in glioma patients. Methods In this multicenter cross-sectional study depression and anxiety were measured with the HADS and subjective cognitive impairment was measured with the MOS-CFS. Univariable logistic regression analyses were performed on all possible confounding predictor variables, and if p-value<0.1, possible confounders were included in the multivariable analyses to identify whether use of AEDs was independently related to depression, anxiety, and/or subjective cognitive impairment.ResultsA total of 272 patients were included. Prevalence of depression differed significantly between patients not using (10%) and using AEDs (21%, unadjusted Odds Ratio [uOR]=2.29 [95%CI=1.05-4.97], p=0.037), but after correction for confounders the statistical significant difference was no longer apparent (adjusted Odds Ratio [aOR]=1.94 [95%CI=0.83-4.50], p=0.125). Prevalences of anxiety (aOR=1.17 [95%CI=0.59-2.29], p=0.659) and subjective cognitive impairment (aOR=0.83 [95%CI=0.34-2.04], p=0.684) did not differ significantly before or after adjustment of confounders between patients not using (19% and 16%, respectively) and using AEDs (26% and 21%, respectively).ConclusionsOur results indicate AED use was not independently associated with concurrent depression, anxiety, or subjective cognitive impairment in glioma patients. Alternative factors seem to have a greater contribution to the risk of having these neuropsychiatric symptoms in glioma patients.


2014 ◽  
Vol 26 (7) ◽  
pp. 1139-1145 ◽  
Author(s):  
Shirin Zaidi ◽  
Martin G. Kat ◽  
Jos F.M. de Jonghe

ABSTRACTBackground:Neuropsychiatric symptoms (NPS) are highly prevalent in dementia. The recently developed Neuropsychiatric Inventory – Clinician rating scale (NPI-C) includes clinical judgment and new symptom domains. Our objective was to evaluate NPI-C reliability and to compare caregiver and clinician ratings across the range of mild to severe cognitive impairment.Methods:This is a cross-sectional observational study. Participants were geriatric memory clinic patients and nursing-home residents (n = 30) with an established diagnosis of dementia or Mild Cognitive Impairment (MCI). A psychiatrist (MK) interviewed caregiver–patient dyads using the NPI-C. Neuropsychological tests and Mini-Mental State Examination (MMSE) were used to assess cognitive impairment. Two NPI-C caregiver interviews were videotaped and rated by psychologists and geriatricians. Intra-class correlations (ICCs) were used to examine inter-rater agreement. Correlation coefficients were calculated to evaluate caregiver and psychiatrist NPI-C ratings. Disagreement between caregiver and clinician was expressed in delta scores and examined across the range of mild to severe cognitive impairment, using Levene's homogeneity of variances tests.Results:Inter-rater agreement on ratings of two caregiver videos was high (ICC = 0.99–1.0). Clinician–caregiver concordance on NPI-C total severity ratings was high (r = 0.77). Variability in clinician–caregiver concordance was associated with cognitive impairment: MMSE (P = 0.02), CAMCOG-R (Cambridge Cognitive Examination-revised) total scores (P = 0.02), CAMCOG-R Memory scores (P = 0.04) and Language scores (P = 0.01).Conclusions:The NPI-C is a reliable measure of NPS in patients with MCI or dementia. Clinician–caregiver agreement on NPS severity may vary with cognitive impairment, underlining the importance of clinician-based measures of NPS.


2015 ◽  
Vol 9 (1) ◽  
pp. 51-57 ◽  
Author(s):  
Allan Gustavo Brigola ◽  
Carlene Souza Silva Manzini ◽  
Gabriel Brassi Silveira Oliveira ◽  
Ana Carolina Ottaviani ◽  
Michelli Pacheco Sako ◽  
...  

The aging process can be accompanied by a slight decline in cognitive functioning, and subjective memory complaints (SMC) appear to be common in the elderly population. OBJECTIVE: To determine whether SMC is associated with cognitive loss or depression and can predict dementia. METHODS: A systematic review of the literature was conducted. Articles were selected on the following databases, LILACS, SCOPUS, SCiELO, PubMed and Web of Science from August to October 2013. Article selection was based on inclusion and exclusion criteria. Studies published between 2010 and 2013, written in English, Spanish or Portuguese, involving populations 65 years or older, were included. Reviews were excluded. RESULTS: After the selection, a summary of the 20 articles retrieved was carried out. Of the total articles retrieved, fifteen were cross-sectional studies and five were longitudinal studies. Most of the cross-sectional studies associated SMC with depression, objective cognitive impairment and anxiety. The emergence of dementia in people with SMC was evidenced in longitudinal studies. Albeit less frequently, SMC were also associated with reduced quality of life, impairment in Activities of Daily Living (ADL), emergence of neuropsychiatric symptoms, lower hippocampal volume, amygdala volume reduction, increased activation of the left temporal, bilateral thalamus, caudate and posterior cingulate, and with the occurrence of ApoE ε4. CONCLUSION: SMC may be associated with changes in mood and/or cognition, and its occurrence appears to increase the likelihood of dementia. In order to further our understanding of the topic, future studies should consider the recruitment of representative samples with control groups and longitudinal designs.


Author(s):  
Pim B. van der Meer ◽  
Johan A. F. Koekkoek ◽  
Martin J. van den Bent ◽  
Linda Dirven ◽  
Martin J. B. Taphoorn

Abstract Introduction AEDs have been associated with depression, anxiety, and cognitive impairment, all frequent complications of glioma and its subsequent treatment, with considerable morbidity and an adverse effect on health-related quality of life. This study aimed to determine the independent association between AED use and self-reported depression, anxiety, and subjective cognitive impairment in glioma patients. Methods In this multicenter cross-sectional study, depression and anxiety were assessed with the HADS and subjective cognitive impairment was assessed with the MOS-CFS. Univariable logistic regression analyses were performed on all potential confounding predictor variables. Potential confounders were included in the multivariable analyses if p-value < 0.1, to evaluate whether use of AEDs was independently related to depression, anxiety, and/or subjective cognitive impairment. Results A total of 272 patients were included. Prevalence of depression differed significantly between patients not using (10%) and using AEDs (21%, unadjusted Odds Ratio [uOR] = 2.29 [95%CI 1.05–4.97], p = 0.037), but after correction for confounders the statistical significant difference was no longer apparent (adjusted Odds Ratio [aOR] = 1.94 [95%CI 0.83–4.50], p = 0.125). Prevalences of anxiety (aOR = 1.17 [95%CI 0.59–2.29], p = 0.659) and subjective cognitive impairment (aOR = 0.83 [95%CI 0.34–2.04], p = 0.684) did not differ significantly before or after adjustment of confounders between patients not using (19% and 16%, respectively) and using AEDs (26% and 21%, respectively). Conclusions Our results indicate AED use was not independently associated with concurrent depression, anxiety, or subjective cognitive impairment in glioma patients. Alternative factors seem to have a greater contribution to the risk of developing neuropsychiatric symptoms in glioma patients.


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