"Cinderella was attacked by the big bad wolf, but the police saved her": intrusions and confabulations on story recall in Korsakoff's syndrome and alcohol-related cognitive impairments.

BACKGROUND: The relation between confabulations and intrusions in patients with Korsakoff's syndrome (KS) and patients with alcohol-related cognitive impairments (ARCI) remains under debate. This study examines (1) differences in the production of confabulations and intrusions between patients with KS and ARCI, (2) whether an altered fairy tale induces more intrusions, and (3) whether different types of intrusions were significantly related to confabulations. METHODS: Twenty-three patients with KS and twenty-two patients with ARCI recalled three different types of stories: a novel story, a fairy tale, and a modified fairy tale. Different types of intrusions were correlated with confabulation measures. RESULTS: Patients with KS produced more intrusions in the modified fairy tale condition than patients with ARCI, but these were unrelated to confabulations. Only unrelated intrusions were related to provoked confabulations. CONCLUSIONS: The results of this study indicate that researchers and clinicians must be aware that in general, intrusions on memory tests should not be interpreted as confabulations. Especially spontaneous confabulations appear to be something completely different from intrusions on any type of story recall. When measuring confabulations it is crucial to use validated instruments.

Behavioral, Emotional and Social Apathy in Alcohol-Related Cognitive Disorders.

Apathy is a fundamental neuropsychiatric symptom of Korsakoff's syndrome (KS) and has also been reported in patients with alcohol use disorder with no (AUD) or less severe cognitive impairments (ARCI). However, research on the nature of apathy is limited in these groups. Aim of this study was to examine the multidimensional nature of apathy in patients with KS, ARCI and AUD. Moreover, we examined differences between apathy ratings by patients and their professional caregivers, and related apathy to everyday functioning and overall cognition. Twenty-five patients with KS, 25 patients with ARCI and 23 patients with AUD participated in this study. Apathy was measured using the apathy motivation index (AMI), which distinguishes behavioral, emotional and social apathy. Both patients and professional caregivers reported social apathy as the most prominent symptom, compared to behavioral and emotional apathy. Apathy ratings did not differ across the three patient groups. Discrepancies between patient and caregiver ratings were observed in patients with KS and ARCI, with more severe apathy reported by caregivers. Caregiver-reported behavioral and social, but not emotional, apathy was related to everyday functioning. These results show that apathy is present in a substantial proportion of patients with alcohol addiction with or without cognitive impairments.

Confabulations in Alcoholic Korsakoff's Syndrome: A Factor Analysis of the Nijmegen-Venray Confabulation List.

Confabulations generally refer to the emergence of memories of experiences and events that, in reality, never took place, and which are unintentionally produced. They are frequently observed in alcoholic Korsakoff's syndrome. The aim of the current study was to validate the Nijmegen-Venray Confabulation List (NVCL), an observation scale for quantifying both spontaneous and provoked confabulations. The NVCL was completed for 252 patients with alcoholic Korsakoff's syndrome. Exploratory and confirmatory factor analyses were conducted to test three- and four-factor models of the NVCL structure. A four-factor model (provoked confabulations, spontaneous confabulations, severity of spontaneous confabulations, and distorted sense of reality) fitted the data better than the initially proposed three-factor model (provoked confabulations, spontaneous confabulations, memory, and orientation). The new instrument is therefore referred to as the NVCL-R. We encourage clinicians to include the assessment of confabulations in the neuropsychological examination, and to do so with validated instruments such as the NVCL-R.

Development and preliminary evaluation of the QUALIKO: an observational quality of life instrument for patients with Korsakoff's syndrome.

BACKGROUND: To develop a Korsakoff-specific measure of quality of life (QoL), to be rated by professional caregivers, and to field-test its psychometric properties in a sample of patients with Korsakoff's syndrome (KS) living in a specialized nursing home. METHODS: A research version of the QUALIKO was developed based on an existing instrument for dementia (the QUALIDEM), literature review and two rounds of surveys among expert professionals involved in the care for patients with KS. Next, QoL was independently rated using the preliminary QUALIKO for 77 patients with KS by two primary caregivers. RESULTS: The research QUALIKO consisted of 48 items describing observable behaviors across ten aspects of QoL relevant to patients with KS. Six items demonstrated poor scalability in the field test. The remaining 42 items all formed subscales with moderate to strong scalability according to Mokken scale analysis. Reliability was acceptable to good across both raters for all subscales (Mokken rho's = 0.70-0.90), except for the two 2-item subscales of negative affect and positive self-image (Mokken rho's = 0.47-0.71). Inter-observer agreement was excellent for five subscales (ICCs = 0.75-0.89) and fair to moderate for the other five subscales (ICCs = 0.59-0.72). The multidimensional internal structure was confirmed and all subscales were significantly correlated with primary caregivers' global ratings of QoL except for positive self-image. Missing item values were low and floor and ceiling effects acceptable for most subscales. CONCLUSIONS: The QUALIKO holds promise as a feasible, reliable, and valid measure of QoL in residential KS patients. Future research in larger samples is needed to confirm the psychometric dimensionality of the instrument, to gather normative data and to examine its test-retest reliability.

The relationship between executive dysfunction and neuropsychiatric symptoms in patients with Korsakoff's syndrome.

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.

Errorless (re)learning of everyday activities in patients with Korsakoff's syndrome: A feasibility study.

Errorless learning has proven to be an effective method for (re)learning tasks in several patient groups with amnesia. However, so far only a handful of studies have examined the effects of errorless learning in patients with Korsakoff's syndrome. The aims of this feasibility study were to (a) examine the effects of errorless learning training on (re)learning tasks in a patient with Korsakoff's syndrome, (b) examine the effects of the nature of the training on the execution of the tasks, and (c) examine characteristics that may mediate learning outcome. Professional caregivers, who were trained in errorless learning principles, taught 51 patients with Korsakoff's syndrome two everyday tasks. Significant improvements in the performance were found after an errorless intervention for different types of trained tasks (activities of daily living, chores, mobility, housekeeping). Moreover, the results of this study suggest that all patients, despite of age, educational level, or level of cognitive functioning, may benefit from errorless learning. The results showed that, despite severe amnesia, patients with Korsakoff's syndrome have the potential to (re)learn everyday skills. Errorless learning might be beneficial for memory rehabilitation in patients with Korsakoff's syndrome in clinical practice. The results of this study are clinically relevant, as successfully learning tasks using errorless learning principles might improve autonomy and independence in the daily lives of patients with Korsakoff's syndrome.

The effect of errorless learning on psychotic and affective symptoms, as well as aggression and apathy in patients with Korsakoff's syndrome in long-term care facilities.

ABSTRACTObjectives:Errorless learning is a promising rehabilitation principle for learning tasks in patients with amnesia, including patients with Korsakoff's syndrome. Errorless learning might possibly also contribute to decreases in behavioral and psychiatric problems, as patients in long-term care facilities become more independent after training. The aim of this study was to examine the effects of errorless learning on potential changes in psychotic and affective symptoms, aggression and apathy, in contrast with a control group who received care as usual. METHODS: The current study is a quasi-experimental study on errorless learning and psychotic and affective symptoms, aggression, and apathy in patients with Korsakoff's syndrome, living in long-term care facilities for patients with Korsakoff's syndrome (KS) in the Netherlands. The GIP-28, HoNOS-ABI, and NVCL-20 were administered to a group of patients with KS who received errorless learning training (n = 51) and a patient control group who received care as usual (n = 31). Wilcoxon Signed-Rank Tests were performed to examine psychotic and affective symptoms, aggression, and apathy at baseline and at follow-up in the errorless learning group and the control group. RESULTS: Errorless learning training effectively reduced psychotic symptoms (including provoked confabulations), affective symptoms, and agitation/aggression. There were no significant changes (increases nor decreases) in the control group. Levels of apathy were stable over time in both groups. CONCLUSIONS: Results with respect to psychotic and affective symptoms, aggression, and apathy are discussed in depth. Patients with KS can become more autonomous in a cared for setting using errorless learning principles. This might also result in decreases in behavioral and psychiatric problems in patients.

Personal semantic and episodic autobiographical memories in Korsakoff syndrome: A comparison of interview methods.

OBJECTIVE: The temporal gradient in patients with Korsakoff's syndrome has been of particular interest in the literature, as many studies have found evidence for a steep temporal gradient, but others have observed more uniform remote memory impairment across all past time periods. Inconsistencies might be the result of the nature of remote memory impairment under study (i.e., nonpersonal or autobiographical memory) and of methodological differences in the examination of remote memory loss. The aim of this study was to examine whether differences between autobiographical memory interview (AMI) and autobiographical interview (AI) procedures influence the presence of a temporal gradient in semantic and episodic autobiographical memory in Korsakoff patients. METHOD: The procedure used in the present study combined the AMI and AI into one study session. We compared the performance of 20 patients with Korsakoff's syndrome and 27 healthy controls. First, participants were asked to recall knowledge from different life periods. Second, participants were asked to recall memories from five life periods. Thirdly, participants were asked to rate their subjective experience of each event recalled on a 5-point scale. Finally, we analyzed the findings in terms of all the memories recalled versus the first memory from each life-period only. RESULTS: Both the AMI and the AI showed a temporally graded retrograde amnesia in the Korsakoff patients for personal semantic and episodic autobiographical memories. The pattern of amnesia in Korsakoff patients was not affected by examining only one event per life-period. Subjective ratings of recalled memories were largely comparable between the groups. CONCLUSIONS: The findings were generally consistent across the AMI and AI. Varying the number of events did not affect the pattern of the gradient. Hence, the temporal gradient in Korsakoff patients is not an artefact of either the AMI or the AI method.

Intrusions and provoked and spontaneous confabulations on memory tests in Korsakoff's syndrome.

INTRODUCTION: Intrusions on verbal memory tests have been used as an index for clinical confabulation. Severe memory impairments in combination with executive dysfunction have been suggested to be the underlying mechanism of confabulation, but to date, this relation is unclear. The aim of this study was (a) to examine the relation between (different types of) intrusions and confabulations in a large sample of confabulating patients with Korsakoff's syndrome (KS) and (b) to investigate whether different measures of executive functioning and memory performance are related to provoked and spontaneous confabulation. METHOD: The Dutch version of the California Verbal Learning Test (CVLT) and various executive function and memory tests were administered to a group of 51 confabulating patients with KS. Professional caregivers rated the severity of provoked and spontaneous confabulation behavior of the patients using the Nijmegen-Venray Confabulation List-20 (NVCL-20). RESULTS: The total number of intrusions on the CVLT was not related to either provoked or spontaneous confabulation scores. None of the CVLT intrusion scores correlated significantly with any of the confabulation scores, but we did find small-to-medium, positive correlations between unrelated intrusions and both provoked confabulations and spontaneous confabulation. Provoked confabulation behavior was associated with executive dysfunction and poorer memory performances. Spontaneous confabulation was not related to performance on measures of executive function and memory. CONCLUSIONS: The total number of intrusions on verbal memory tests and clinical confabulations appear to be different phenomena. Only unrelated intrusions produced on the CVLT might possibly be related to confabulations. The production of provoked, but not spontaneous, confabulation is associated with executive dysfunction and memory deficits.

Assessment of Confabulation in Patients with Alcohol-Related Cognitive Disorders: The Nijmegen-Venray Confabulation List (NVCL-20).

OBJECTIVE: Even though the first awareness of confabulations is often based on observations, only questionnaires and structured interviews quantifying provoked confabulations are available. So far, no tools have been developed to measure spontaneous confabulation. This study describes and validates an observation scale for quantifying confabulation behavior, including spontaneous confabulations, in clinical practice. METHOD: An observation scale consisting of 20 items was developed, the Nijmegen-Venray Confabulation List-20 (NVCL-20). This scale covers spontaneous confabulation, provoked confabulation, and memory and orientation. Professional caregivers completed the NVCL-20 for 28 Korsakoff (KS) patients and 24 cognitively impaired chronic alcoholics (ALC). Their ratings were related to the Dalla Barba Confabulation Battery (DBCB), Provoked Confabulation Test (PCT), and standard neuropsychological tests. RESULTS: The categories of the NVCL-20 have "good" to "excellent" internal consistency and inter-rater agreement. The KS patients confabulated more (both spontaneously and provoked), and more memory and orientation problems were observed. Correlations with neuropsychological test scores showed that confabulations were associated with memory deficits, but not with intrusions or tests of executive dysfunction. CONCLUSIONS: The NVCL-20 is the first instrument that includes items addressing spontaneous confabulation. Administration is reliable, valid and feasible in clinical practice, making it a useful addition to existing confabulating measures.