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.

Assessing deictic relational responding in psychosis using the implicit relational assessment procedure

The current study sought to investigate perspective-taking in a group of individuals diagnosed with psychosis. The Psychosis Implicit Relational Assessment Procedure (Psychosis-IRAP) contrasted statements and questions referring to 'psychotic' and 'non-psychotic' experiences. The IRAP and a Faux-pas (Theory of Mind) test were presented to two groups of participants: a clinical group with a diagnosis of psychosis and a control group. IRAP effects for each group were in the predicted direction and a ROC analysis showed that the IRAP correctly classified 80% of the individuals with psychosis with a sensitivity level of 84.2% and a specificity level of 27.8%. The IRAP was thus successfully used to correctly classify the population of clinical individuals. However, the study also demonstrated that the two groups were similar with regard to their level of competency on the perspective-taking IRAP. The article also discusses the relationship between the data and recent developments in RFT, in terms of relational flexibility

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Assessing deictic relational responding in social anxiety using the implicit relational assessment procedure

The current study sought to investigate perspective-taking in a group of individuals diagnosed with psychosis. The Social Anxiety Implicit Relational Assessment Procedure (SA-IRAP) contrasted statements and questions referring to 'anxious' and 'non-anxious' experiences. The SA-IRAP and a Faux-pas (Theory of Mind) test were presented to two groups of participants: a clinical group with a diagnosis of social anxiety and a group of controls. IRAP effects for each group were in the predicted direction and a ROC analysis showed that the IRAP correctly classified 77% of the individuals with social anxiety with a sensitivity level of 97% and a specificity level of 36%. The IRAP was thus successfully used to correctly classify the sample of clinical individuals. However, the study also demonstrated that the two groups were similar with regard to their level of competency on the perspective-taking IRAP. The article also discusses the relationship between the data and recent developments in RFT

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Differentiation between defensive personality functioning and psychopathology as measured by the DSQ-42 and MMPI-2-RF

Defensive functioning is considered one of the core aspects of personality functioning and its maturity level is regarded an important predictor of psychopathology and more specific personality pathology. The current investigation assesses the relation between overall defensive functioning, as measured by the Defense Style Questionnaire-42 (DSQ-42), and higher order models of psychopathology as measured by the Minnesota Multiphasic Personality Inventory-2-Restructured Form (MMPI-2-RF). The DSQ-42 and MMPI-2-RF was completed by 383 patients. We analysed the MMPI-2-RF personality and psychopathology as measured with the Restructured Clinical scales and the Personality Psychopathology Five-revised scales using Goldberg’s Bass Ackwards Method. Higher order dimensions of personality and psychopathology in the current investigation demonstrated structural similarity with previously reported higher order models. Next we examined the optimal level of differentiation of defensive functioning, as measured by the DSQ-42 Total and Overall Defensive Functioning scores, to personality and psychopathology at each succeeding level of the hierarchical factor structures. Results indicated that immature defense mechanisms exemplify strong correlations with internalizing pathology (i.e., Demoralisation and Introversion), but not with externalizing pathology and thought disorder. The differentiation of defensive functioning from higher order models of psychopathology and maladaptive personality traits seems to be limited, based on the current results. The DSQ-42 appeared to have a large overlap and correlations with internalizing pathology, which appeared to be due to its item content: mostly intrapsychic and immature defenses. Theoretical and clinical implications considering the use of the DSQ are discussed

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The effect of errorless learning on quality of life in patients with Korsakoff's syndrome.

BACKGROUND: Errorless learning (EL) is a promising rehabilitation principle for (re)learning instrumental activities in patients with amnesia, including patients with Korsakoff's syndrome (KS). Successfully (re)learning tasks might improve the sense of competence and independence, and subsequently the quality of life. Quality of life in patients with KS has received limited attention, and no studies have been conducted to experimentally examine the effect of EL on quality of life in patients in this patient group. MATERIALS AND METHODS: The QUALIDEM, an observation scale for quality of life, was completed by professional nurses before and after EL training in a group of fifty-one patients with KS. This scale was also completed for a group of thirty-one control patients receiving care as usual but no EL training. RESULTS: Quality of life was significantly increased on eight of the nine subscales in the Korsakoff group who participated in an EL training. There was a trend toward a significant increase in "positive affect" (ie, the ninth subscale). In contrast, no changes over time were found on any of the subscales in the control group that did not participate in any EL training. CONCLUSION: Despite severe memory impairments, patients with KS still have the potential to (partially) (re)learn tasks using EL. This potential should be exploited, as the successes of (re)-learning might improve the quality of life of Korsakoff patients in nursing homes.

Psychometric properties of the Dutch Anxiety Change Expectancy Scale (ACES-NL)

Research demonstrates that patients’ expectations about treatment outcome are an important predictor of actual psychotherapeutic treatment outcome. So far, only few psychometrically sound expectancy questionnaires are available to assess treatment expectancy. The Anxiety Change Expectancy Scale (ACES) is a promising 20-item questionnaire which measures patients’ expectations about being able to change anxiety regarding and regardless from treatment. Psychometric properties of the Dutch language version of the ACES (ACES-NL) are investigated in 212 patients referred to a mental health institute because of suspected anxiety disorders. Reliability (i.e., internal consistency, inter-item, test-retest reliability) is investigated. Validity is examined in terms of internal structure and relations with the Minnesota Multiphasic Personality Inventory-2-Restructured Form (MMPI-2-RF), Beck Hopelessness Scale (BHS) and Self-Efficacy Scale (SES). Reliability statistics are good to excellent. Factor analysis reveals a one factor solution. Meaningful relations with relevant MMPI-2-RF scales are established. BHS and SES scores demonstrate satisfactory concurrent and discriminant validity. Treatment outcome expectancy is discussed against the background of relevant models. Further research on predictive validity of the ACES-NL is warranted (AU)

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Cycloid psychoses in the psychosis spectrum: evidence for biochemical differences with schizophrenia.

Cycloid psychoses (CP) differ from schizophrenia regarding symptom profile, course, and prognosis and over many decades they were thought to be a separate entity within the psychosis spectrum. As to schizophrenia, research into the pathophysiology has focused on dopamine, brain-derived neurotrophic factor, and glutamate signaling in which, concerning the latter, the N-methyl-d-aspartate receptor plays a crucial role. The present study aims to determine whether CP can biochemically be delineated from schizophrenia. Eighty patients referred for psychotic disorders were assessed with the Comprehensive Assessment of Symptoms and History, and (both at inclusion and after 6 weeks of antipsychotic treatment) with the Positive and Negative Syndrome Scale and Clinical Global Impression. From 58 completers, 33 patients were diagnosed with schizophrenia and ten with CP according to the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition, and Leonhard criteria, respectively. Fifteen patients were diagnosed with other disorders within the psychosis spectrum. At both time points, blood levels of the dopamine metabolite homovanillic acid, brain-derived neurotrophic factor, and amino acids related to glutamate neurotransmission were measured and compared with a matched control sample. Patients with CP showed a significantly better response to antipsychotic treatment as compared to patients with schizophrenia. In CP, glycine levels were elevated and tryptophan levels were lowered as compared to schizophrenia. Glutamate levels were increased in both patient groups as compared to controls. These results, showing marked differences in both treatment outcome and glutamate-related variable parameters, may point at better neuroplasticity in CP, necessitating demarcation of this subgroup within the psychosis spectrum.

Measuring illness insight in patients with alcohol-related cognitive dysfunction using the Q8 questionnaire: a validation study.

AIM: Impaired illness insight may hamper treatment outcome in patients with alcohol-related cognitive deficits. In this study, a short questionnaire for the assessment of illness insight (eg, the Q8) was investigated in patients with Korsakoff's syndrome (KS) and in alcohol use disorder (AUD) patients with mild neurocognitive deficits. METHODS: First, reliability coefficients were computed and internal structure was investigated. Then, comparisons were made between patients with KS and patients with AUD. Furthermore, correlations with the Dysexecutive Questionnaire (DEX) were investigated. Finally, Q8 total scores were correlated with neuropsychological tests for processing speed, memory, and executive function. RESULTS: Internal consistency of the Q8 was acceptable (ie, Cronbach's α =0.73). The Q8 items represent one factor, and scores differ significantly between AUD and KS patients. The Q8 total score, related to the DEX discrepancy score and scores on neuropsychological tests as was hypothesized, indicates that a higher degree of illness insight is associated with a higher level of cognitive functioning. CONCLUSION: The Q8 is a short, valid, and easy-to-administer questionnaire to reliably assess illness insight in patients with moderate-to-severe alcohol-related cognitive dysfunction.

Absence epilepsy and the CHD2 gene: an adolescent male with moderate intellectual disability, short-lasting psychoses, and an interstitial deletion in 15q26.1-q26.2.

Deletions of the 15q26 region encompassing the chromodomain helicase DNA binding domain 2 (CHD2) gene have been associated with intellectual disability, behavioral problems, and several types of epilepsy. Including the cases mentioned in ECARUCA (European cytogeneticists association register of unbalanced chromosome aberrations) and DECIPHER (database of genomic variation and phenotype in humans using ensembl resources), so far, a total of 13 intellectually disabled patients with a genetically proven deletion of the CHD2 gene are described, of whom eleven had a history of severe forms of epilepsy starting from a young age. In this article, a moderately intellectually disabled 15-year-old male with a 15q26.1-q26.2 interstitial deletion is reported, who was referred for analysis of two recent short-lasting psychotic episodes that were nonresponsive to antipsychotic treatment and recurrent disinhibited behaviors since early infancy. Careful interdisciplinary assessment revealed that the psychotic phenomena originated from a previously unrecognized absence epilepsy. Treatment with valproic acid was started which resulted in full remission of psychotic symptoms, and consequently, substantial improvement of behavior. It was concluded that in case of (rare) developmental disorders with genetically proven etiology, a detailed inventory of anamnestic data and description of symptomatology over time may elucidate epilepsy-related psychopathology for which a specific treatment regimen is needed.

Applicability of the Rivermead Behavioural Memory Test - Third Edition (RBMT-3) in Korsakoff's syndrome and chronic alcoholics.

PURPOSE: To examine the applicability of the newly developed Rivermead Behavioural Memory Test - Third Edition (RBMT-3) as an ecologically-valid memory test in patients with alcohol-related cognitive disorders. PATIENTS AND METHODS: An authorized Dutch translation of the RBMT-3 was developed, equivalent to the UK version, and administered to a total of 151 participants - 49 patients with amnesia due to alcoholic Korsakoff's syndrome, 49 patients with cognitive impairment and a history of chronic alcoholism, not fulfilling the Korsakoff criteria, and 53 healthy controls. Between-group comparisons were made at subtest level, and the test's diagnostic accuracy was determined. RESULTS: Korsakoff patients performed worse than controls on all RBMT-3 subtests (all P-values < 0.0005). The alcoholism group performed worse than controls on most (all P-values < 0.02), but not all RBMT-3 subtests. Largest effects were found between the Korsakoff patients and the controls after delayed testing. The RBMT-3 had good sensitivity and adequate specificity. CONCLUSION: The RBMT-3 is a valid test battery to demonstrate everyday memory deficits in Korsakoff patients and non-Korsakoff patients with alcohol abuse disorder. Korsakoff patients showed an impaired performance on subtests relying on orientation, contextual memory and delayed testing. Our findings provide valuable information for treatment planning and adjustment in patients with alcohol-related cognitive impairments.

Cycloid psychoses: Leonhard's descriptions revisited

Background and Objectives: Cycloid psychoses are characterized by polymorphic symptomatology with intraphasic bipolarity, a remitting and recurrent course and favourable prognosis. Perris and Brockington (P&B) described the first set of operational criteria that were partly incorporated in ICD-10. The present study investigates psychopathological profiles according to the P&B criteria and the original descriptions by Leonhard, both against the background of the criteria from the prevailing international classification systems. Methods: Eighty patients with psychotic disorders were recruited and assessed with various psychometric instruments at baseline and after six weeks of antipsychotic treatment in order to investigate the presence of cycloid psychoses according to Leonhard (LCP) and the effect of treatment with antipsychotics. The overlap between LCP and DSM-IV Brief Psychotic Disorder (BPD), ICD Acute Polymorphic Psychotic Disorder (APP) and P&B criteria was calculated. Results: Using P&B criteria and a symptom checklist adapted from the original descriptions by Leonhard, 14 and 12 cases of cycloid psychosis were identified respectively reflecting a prevalence of 15-18%. Small though significant concordance rates were found between LCP and both DSM-BPD and ICD-APP. Concordance between LCP and P&B criteria was also significant, but modest. Conclusions: This study demonstrates that LCP can be identified in a substantial number of patients with psychotic disorders. Cycloid psychoses are not adequately covered in current classification systems and criteria. Since they are demonstrated to have a specific psychopathological profile, relapsing course and favourable prognosis, it is advocated to include these psychoses in daily differential diagnostic procedures (AU)

Copy number variants in a sample of patients with psychotic disorders: is standard screening relevant for actual clinical practice?

With the introduction of new genetic techniques such as genome-wide array comparative genomic hybridization, studies on the putative genetic etiology of schizophrenia have focused on the detection of copy number variants (CNVs), ie, microdeletions and/or microduplications, that are estimated to be present in up to 3% of patients with schizophrenia. In this study, out of a sample of 100 patients with psychotic disorders, 80 were investigated by array for the presence of CNVs. The assessment of the severity of psychiatric symptoms was performed using standardized instruments and ICD-10 was applied for diagnostic classification. In three patients, a submicroscopic CNV was demonstrated, one with a loss in 1q21.1 and two with a gain in 1p13.3 and 7q11.2, respectively. The association between these or other CNVs and schizophrenia or schizophrenia-like psychoses and their clinical implications still remain equivocal. While the CNV affected genes may enhance the vulnerability for psychiatric disorders via effects on neuronal architecture, these insights have not resulted in major changes in clinical practice as yet. Therefore, genome-wide array analysis should presently be restricted to those patients in whom psychotic symptoms are paired with other signs, particularly dysmorphisms and intellectual impairment.

Phelan-McDermid syndrome in two adult brothers: atypical bipolar disorder as its psychopathological phenotype?

The 22q13.3 deletion, or Phelan-McDermid syndrome, is characterized by global intellectual disability, generalized hypotonia, severely delayed or absent speech associated with features of autism spectrum disorder, and minor dysmorphisms. Its behavioral phenotype comprises sleep disturbances, communication deficits, and motor perseverations. Data on psychological dysfunctions are so far not available. Previous studies have suggested that the loss of one copy of the gene SH3 and multiple ankyrin repeat domains 3 (SHANK3) is related to the neurobehavioral phenotype. Additional genes proximal to SHANK3 are also likely to play a role in the phenotype of patients with larger deletions. The present paper describes two adult brothers with an identical 2.15 Mb 22qter (22q13.32q13.33) deletion, of whom the youngest was referred for evaluation of recurrent mood changes. In both patients, magnetic resonance imaging of the brain showed hypoplasia of the vermis cerebelli. Extensive clinical examinations led to a final diagnosis of atypical bipolar disorder, of which symptoms fully remitted during treatment with a mood stabilizer. In the older brother, a similar psychopathological picture appeared to be present, although less severe and with a later onset. It is concluded that the behavioral phenotype of the 22q13.3 deletion syndrome comprises absent or delayed speech and perseverations with associated autistic-like features, whereas its psychopathological phenotype comprises an atypical bipolar disorder. The latter may have implications for the treatment regime of the syndrome-related behavioral disturbances.

Recurrent major depression, ataxia, and cardiomyopathy: association with a novel POLG mutation?

At present, more than 100 disease mutations in mitochondrial DNA polymerase γ (POLG) have been indentified that are causally related to an array of neuropsychiatric diseases affecting multiple systems. Both autosomal recessive and autosomal dominant forms can be delineated, the latter being associated with Parkinsonism and depressive or psychotic syndromes. In this report, a middle-aged female patient with recurrent major depression with melancholic features, slowly progressive gait instability, and dilated cardiomyopathy is described. Detailed diagnostic evaluation was performed to elucidate the supposed relationship between ataxia, cardiomyopathy, and major depression with melancholia. After extensive genetic and metabolic investigation, a nucleotide substitution c.2207 A→G in the POLG gene resulting in amino acid change Asn 736Ser in exon 13 was demonstrated. This mutation was considered to be compatible with a mitochondrial disorder and implicated in the pathophysiology of the neuropsychiatric syndrome. It is concluded that this novel POLG mutation forms the most parsimonious etiological explanation for the here-described combination of ataxia, major depression, and cardiomyopathy. Therefore, in patients with a complex neuropsychiatric presentation, extensive diagnostic analysis is warranted, including the search for mitochondriopathies, in order to avoid unnecessary delay of adequate treatment.

Celeration of Executive Functioning while Solving the Tower of Hanoi: Two Single Case Studies Using Protocol Analysis

The present study was designed to systematically explore individual learning abilities by an in depth analysis of the performances of two single participants on the five disk Tower of Hanoi. Verbal protocols were obtained to provide us with a detailed picture of strategy development and learning. Correct and incorrect responses were plotted on a Standard Celeration Chart in order to analyze the growth of learning across time (i.e., celeration). Protocol analysis revealed that both participants formulated rules, indicating the presence of previously learned problem-solving strategies or “schemas,” but also showed a substantial difference in adequately adapting these rules to modify their executive behaviour. Furthermore, celeration trends indicated a markedly increase of speed and accuracy level for performances of participant A, whereas performances of participant B showed no (significant) changes in behaviour accuracy. We concluded that a systematic investigation of the actual processes that underlie the acquisition of strategies using protocol analysis, can provide us with a detailed picture of individual differences on EF. Additionally, the Standard Celeration Chart proved to be a good assessment method to monitor behavioural changes and individual learning. On the critical side, further investigation of the differential effects of age, gender and pathology on individual performance seems to be warranted (AU)

El presente estudio se diseñó para explorar sistemáticamente habilidades de aprendizaje individual mediante un profundo análisis de las ejecuciones de dos participantes en los cinco discos de la Torre de Hanoi. Se obtuvieron protocolos verbales para proporcionar una detallada imagen del desarrollo de estrategias y aprendizaje. Las respuestas correctas e incorrectas se dispusieron sobre un gráfico de aceleración en order a analizar el creci- miento del apredizaje a lo largo del tiempo. El análisis del protocolo reveló que ambos participantes formularon reglas, indicando la presencia de estrategias de solución de problemas previamente aprendidas o “esquemas”, pero también mostraron una diferencia sustancial en adaptar adecuadamente estas reglas para modificar su ejecución. Además, las tendencias de aceleración indican un marcado incremento de la velocidad y del nivel de precisión en las ejecuciones del participante A, mientras que las ejecuciones del participante B no mostraron cambios significativos en la precisión. Se concluye que la investigación sistemática de los procesos que subyacen la adquisición de estrategias usando análisis de protocolos, puede proporcionar una imagen detallada de las diferencias individuales en funcionamiento ejecutivo. Adicionalmente, el empleo de la Standard Celeration Chart prueba ser un buen método de evaluación para observar los cambios conductuales y el aprendizaje individual. Son necesarios nuevos estudios sobre los efectos diferenciales de la edad, el sexo y la presencia de patologías (AU)