scholarly journals Classical galactosemia: neuropsychological and psychosocial functioning beyond intellectual abilities

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Mendy M. Welsink-Karssies ◽  
Kim J. Oostrom ◽  
Merel E. Hermans ◽  
Carla E. M. Hollak ◽  
Mirian C. H. Janssen ◽  
...  
Keyword(s):  
Cognitive Functioning ◽  
Social Functioning ◽  
Internalizing Problems ◽  
Psychosocial Functioning ◽  
Cognitive Test ◽  
Large Variability ◽  
Classical Galactosemia ◽  
Cognitive Domains ◽  
Neuropsychological Profile ◽  
Intellectual Abilities

Abstract Background Despite early diagnosis and treatment, Classical Galactosemia (CG) patients frequently develop long-term complications, such as cognitive impairment. Available literature primarily reports on general intellectual abilities and shows a substantially lower Full Scale Intelligence Quotient (FSIQ) in CG patients than in the general population. Both problems in social functioning as well as internalizing problems are often reported in CG patients. The combination of intelligence, cognitive functioning, behavior and social functioning has not been studied systematically in CG patients. Methods To determine if CG patients demonstrate a specific neuropsychological and psychosocial profile, we investigated intelligence, functioning on multiple cognitive domains, behavior and social functioning with a comprehensive neuropsychological test battery and questionnaires (self- and proxy-reported). Results The data of 48 patients, aged 4–47 years are reported. FSIQ ranged from 45 to 103 (mean 77 ± 14). A negative correlation between age and FSIQ was demonstrated (p = 0.037) which resulted directly from the inclusion of four young ‘milder’ patients detected by newborn screening (NBS) with an expected better clinical outcome. Compared to normative data, patients had significantly lower but highly variable scores on all cognitive domains, especially on tests requiring mental speed. In the context of the FSIQ, 43% of the cognitive test results exceeded IQ based expectations. Overall, the patients’ scores on social functioning were in the normal range but internalizing problems were frequently reported. In our cohort, an early initiation of dietary treatment due to NBS or family screening did not result in a more favorable neuropsychological outcome. Conclusions In this study, we demonstrated that as a cohort, CG patients have a below average intelligence and impaired cognitive functioning without a distinctive neuropsychological profile. The effect of age on neurocognitive functioning should be assessed in longitudinal studies. Social functioning was not impaired, but patients may be at risk for internalizing problems. Considering the large variability in cognitive, behavioral and social functioning and the finding that cognitive outcomes may exceed IQ based expectations, an individual evaluation and follow-up is warranted in all CG patients to ensure timely support if needed.

Assessment of cognitive impairment with the cognitive assessment interview (CAI) was useful for identifying poor psychosocial functioning outcome in patients with psychosis

2016 ◽  
Vol 33 (S1) ◽  
pp. S106-S107
Author(s):  
A. Sánchez-Torres ◽  
M.R. Elosúa ◽  
R. Lorente-Omeñaca ◽  
L. Moreno-Izco ◽  
V. Peralta ◽  
...  
Keyword(s):  
Social Cognition ◽  
Cognitive Functioning ◽  
Verbal Memory ◽  
Psychosocial Functioning ◽  
Cognitive Assessment ◽  
Daily Functioning ◽  
Everyday Functioning ◽  
Cognitive Domains ◽  
Assessment Interview ◽  
Competing Interest

IntroductionCognitive impairments clearly impact the daily functioning of patients with psychosis.ObjectivesTo assess cross-sectionally whether there are differences in the cognitive domains assessed with the CAI, for considering the real-world functioning of a sample of patients with psychosis.MethodsThe sample consisted of 76 patients with a DSM-IV psychotic disorder. Patients were assessed with the cognitive assessment interview (CAI), which is an interview-based measure of cognitive functioning that is intermediate between cognitive functioning and daily functioning, and three subscales of the specific levels of functioning (SLOF), an informant-rated measure of functioning. The CAI was used to assess the patient and an informant, and these scores were integrated into a rater composite score. We divided the sample by a median-split procedure for each of the three functional domains, and then applied ANOVAs to compare the two groups (impaired/not impaired) in the six cognitive domains of the CAI: working memory, attention, verbal memory, problem solving, processing speed, and social cognition.ResultsWe found significant differences between the impaired vs. non-impaired groups in most of the cognitive domains assessed with the CAI (Fig. 1).ConclusionsInterview-based assessment of cognition with the CAI allows for the prediction of everyday functioning in patients with psychosis. Impairment in almost all CAI cognitive domains, except for social cognition, was associated with poorer psychosocial functioning.Disclosure of interestThe authors have not supplied their declaration of competing interest.

scholarly journals Correction to: Classical galactosemia: neuropsychological and psychosocial functioning beyond intellectual abilities

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Mendy M. Welsink-Karssies ◽  
Kim J. Oostrom ◽  
Merel E. Hermans ◽  
Carla E. M. Hollak ◽  
Mirian C. H. Janssen ◽  
...  
Keyword(s):  
Psychosocial Functioning ◽  
Classical Galactosemia ◽  
Intellectual Abilities

An amendment to this paper has been published and can be accessed via the original article.

Abstract WP190: Development of a Comprehensive Neuropsychological Battery to Assess Post-Stroke Cognitive Functioning

Stroke ◽  
2020 ◽  
Vol 51 (Suppl_1) ◽  
Author(s):  
Lauren Drag ◽  
Nima Aghaeepour ◽  
Michael Mlynash ◽  
Elizabeth Osborn ◽  
Esther Rah ◽  
...  
Keyword(s):  
Cognitive Functioning ◽  
Processing Speed ◽  
Test Battery ◽  
Cognitive Domain ◽  
Cognitive Test ◽  
Fine Motor ◽  
Neuropsychological Battery ◽  
Cognitive Domains ◽  
Post Stroke ◽  
Prospective Longitudinal

Introduction: Cognitive outcomes vary widely following stroke and there is need for further research identifying the trajectory of post-stroke cognitive functioning. This requires a neuropsychological test battery that takes into account the unique challenges of testing cognition post-stroke (e.g., aphasia, hemiparesis) and that is relatively brief yet assesses cognition across multiple domains. The aim of this study was to develop such a battery and describe its characteristics. Methods: StrokeCog is a prospective longitudinal cohort study examining cognitive trajectories following stroke. A 60-minute neuropsychological battery including 10 tests (yielding 17 neuropsychological variables) was administered to 86 participants 6-12 months after stroke. Raw scores for each of the 17 variables were transformed to age-corrected z-scores. A pair-wise undirected Pearson correlation graph was created using all available variables to visualize the correlation network representing the variables and to identify the cognitive domains assessed by these variables. Results: Participants ranged in age from 26-87 (M = 63.85, SD = 12.58); 59% of the sample was male. Sixteen cognitive variables loaded onto five cognitive domains: memory, expressive language, processing speed, visuospatial functioning, and fine motor functioning. 62% of participants demonstrated impaired cognition in at least one domain with 22% demonstrating impairment in 2 or more domains. Memory and processing speed were most commonly impaired (45% and 53% of participants, respectively). Visuospatial functioning was least impacted (14% of participants). Conclusions: A comprehensive assessment of cognition in post-stroke patients can be obtained using a 60-minute cognitive test battery, allowing repeated annual assessments to elucidate cognitive trajectories post-stroke. The results show a high rate of cognitive impairment in at least one cognitive domain among the participants.

scholarly journals Long-term trajectory of cognitive performance in people with bipolar disorder and controls: 6-year longitudinal study

BJPsych Open ◽  
2021 ◽  
Vol 7 (4) ◽  
Author(s):  
Timea Sparding ◽  
Erik Joas ◽  
Caitlin Clements ◽  
Carl M. Sellgren ◽  
Erik Pålsson ◽  
...  
Keyword(s):  
Bipolar Disorder ◽  
Cognitive Functioning ◽  
Cognitive Performance ◽  
Repeated Measures ◽  
Cognitive Test ◽  
Healthy Controls ◽  
Cross Sectional ◽  
Time Interaction

Background Cross-sectional studies have found impaired cognitive functioning in patients with bipolar disorder, but long-term longitudinal studies are scarce. Aims The aims of this study were to examine the 6-year longitudinal course of cognitive functioning in patients with bipolar disorder and healthy controls. Subsets of patients were examined to investigate possible differences in cognitive trajectories. Method Patients with bipolar I disorder (n = 44) or bipolar II disorder (n = 28) and healthy controls (n = 59) were tested with a comprehensive cognitive test battery at baseline and retested after 6 years. We conducted repeated measures ANCOVAs with group as a between-subject factor and tested the significance of group and time interaction. Results By and large, the change in cognitive functioning between baseline and follow-up did not differ significantly between participants with bipolar disorder and healthy controls. Comparing subsets of patients, for example those with bipolar I and II disorder and those with and without manic episodes during follow-up, did not reveal subgroups more vulnerable to cognitive decline. Conclusions Cognitive performance remained stable in patients with bipolar disorder over a 6-year period and evolved similarly to healthy controls. These findings argue against the notion of a general progressive decline in cognitive functioning in bipolar disorder.

scholarly journals A-102 The Relationship Between Retinal and Cognitive Functioning in Schizophrenia

2020 ◽  
Vol 35 (6) ◽  
pp. 895-895
Author(s):  
Demmin D ◽  
Klein S ◽  
Silverstein S
Keyword(s):  
Cognitive Functioning ◽  
Executive Control ◽  
Cell Structure ◽  
Cell Activity ◽  
Amacrine Cells ◽  
Structure And Function ◽  
Cognitive Test ◽  
Retinal Cell ◽  
Control Processes ◽  
And Function

Abstract Objective The retina may provide a unique window into brain structure and function as an accessible part of the central nervous system. Abnormalities in retinal cell structure and function have been associated with brain pathology (e.g., brain volume loss, cognitive impairment) in several neuropsychiatric disorders (e.g., MS, Alzheimer’s disease, Parkinson’s disease). A number of prior studies using flash electroretinography (fERG) have reported reduced retinal cell activity in schizophrenia (SZ). Impairments in cognitive functioning are a core feature of SZ and deficits in executive control processes involving prefrontal cortex (PFC) activity are strong predictors of functional capacity. This study examined whether retinal cell functioning is related to brain function, as indexed by cognitive function, in SZ, and if these relationships were stronger in particular domains (e.g., PFC dependent functions vs. less PFC dependent functions). Method Twenty-six SZ participants and 24 healthy controls (HC) completed fERG and cognitive testing. fERG measurements included a-wave (photoreceptor cells), b-wave (bipolar-Müller cells), and oscillatory potential (OP; amacrine cells) amplitudes and implicit times. Cognitive tests assessed executive control processes (i.e., attention/processing speed, behavior initiation, response inhibition, working memory), and non-executive control processes (i.e., emotion recognition/discrimination). Results In the HC group, a-wave amplitude was correlated with cognitive test scores and OP amplitude was related to cognitive performance in the SZ group. However, overall, retinal cell activity did not appear to be strongly related to scores on cognitive tasks, regardless of whether or not they involved frontal brain regions. Conclusion Impairments in retinal and cognitive functioning may reflect distinct disease mechanisms in schizophrenia.

scholarly journals Cognitive Functioning in Patients with Pediatric-Onset Multiple Sclerosis, an Updated Review and Future Focus

Children ◽  
2019 ◽  
Vol 6 (2) ◽  
pp. 21 ◽  
Author(s):  
Joy B. Parrish ◽  
Emily Fields
Keyword(s):  
Multiple Sclerosis ◽  
Cognitive Functioning ◽  
Psychosocial Functioning ◽  
Functional Outcomes ◽  
Daily Living ◽  
Demyelinating Diseases ◽  
Future Studies ◽  
Preventative Measures ◽  
The Impact ◽  
Pediatric Onset

Pediatric-onset multiple sclerosis (POMS) is relatively rare, but as technology and neuroimaging advance, an increasing number of cases are identified, and our understanding of how multiple sclerosis (MS) impacts the developing brain improves. There are consistent findings in the literature highlighting the impact of MS and other demyelinating diseases on cognitive functioning and cognitive development. We also have a better understanding of how POMS impacts psychosocial functioning and functional outcomes in daily living. This paper hopes to review findings associated with cognitive and psychosocial functioning in patients with POMS, as well as explore more recent advances in the field and how they relate to cognitive and psychosocial outcomes. We also discuss the ongoing need for future studies with a focus on better understanding deficits and disease correlates, but also preventative measures and potential rehabilitation.

Psychosocial functioning in relation to symptomatic remission: A longitudinal study of first episode schizophrenia

2015 ◽  
Vol 30 (8) ◽  
pp. 907-913 ◽  
Author(s):  
K. Jaracz ◽  
K. Górna ◽  
J. Kiejda ◽  
B. Grabowska-Fudala ◽  
J. Jaracz ◽  
...  
Keyword(s):  
Social Functioning ◽  
Psychosocial Functioning ◽  
Early Stage ◽  
First Episode ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Poor Outcome ◽  
Remission Status ◽  
Symptomatic Remission

AbstractObjectivesThe aims of the study were: (1) to evaluate longitudinally symptomatic remission in first-episode (FE) schizophrenia, (2) to describe symptoms, social functioning and quality of life (Qol) in relation to remission status, and (3) to determine the long-term outcome of schizophrenia and its early predictors.MethodsSixty-four patients were assessed 1 month after a first hospitalization (T1), 12 months (T2), 4–6 years (T3), and 7–11 years (T4) after T1. The patients were allocated to three remission groups according to their remission status over the whole observation period, e.g. stable remission (SR), unstable remission (UR) and non-remission (NR). The PANSS, Social Functioning Scale and WHOQoL were used to evaluate the patients’ psychosocial functioning levels, symptomatic and functional remissions and satisfying QoL. A good outcome was defined as meeting, simultaneously, the criteria of symptomatic and functional remissions and satisfying QoL at T4, while failure to meet all of these criteria was defined as a poor outcome.ResultsAmong them, 17.2% patients were in stable remission, 57.8% in unstable remission and 25.0% were unremitted at all time points. The SR group had lower levels of psychopathological symptoms and reported better social functioning and QoL than the NR group. During the follow-up, the symptoms increased, social functioning slightly improved and QoL did not change. At T4, 53% of the sample had a poor outcome, which was independently predicted by the longer duration of untreated psychosis and a lack of satisfying QoL at T1.ConclusionsOur results demonstrate that: (1) the long-term course in schizophrenia is heterogeneous and that three illness trajectories exist, (2) social functioning and QoL are only partially connected with symptomatic remission (3), the risk of a poor outcome may potentially be reduced by appropriate interventions at an early stage of the illness.

Emotion processing and social functioning in euthymic bipolar disorder

2016 ◽  
Vol 33 (S1) ◽  
pp. S125-S126
Author(s):  
A. Aparicio ◽  
E.M. Sánchez-Morla ◽  
J.L. Santos ◽  
J. Mateo
Keyword(s):  
Social Functioning ◽  
Psychosocial Functioning ◽  
Emotion Processing ◽  
Bipolar Disorders ◽  
Poor Performance ◽  
Premorbid Intelligence ◽  
Partial Correlations ◽  
Understanding Emotions ◽  
Competing Interest ◽  
Bipolar Patients

IntroductionA large number of studies have found that patients with bipolar disorders have a poor performance in tasks assessing social cognition.Objectives and aimsThe present study aimed to investigate whether euthymic bipolar patients (EBP) have a dysfunction in emotion processing when compared to controls. An additional objective was to determine whether there is association between emotion processing and psychosocial functioning.MethodsA sample of 53 EBP and 53 healthy controls matched for age, gender, education level and premorbid intelligence were studied. All subjects were assessed using the MATRICS Consensus Cognitive Battery (MCCB) and two additional executive function measures: the Trail Making Test–Part B and the Stroop Test. Emotion processing was examined using the Mayer-Salovey-Caruso Emotional Intelligence Test (MSCEIT). Psychosocial functioning was assessed using the Global Assessment of Functioning (GAF) scale and the Functional Assessment Short Test (FAST).ResultsFor the MSCEIT, EBP obtained lower total scores (P = 0.001), experiential area scores (P = 0.012), strategic area scores (P = 0.000), perceiving emotions branch scores (P = 0.008), understanding emotions branch scores (P = 0.014) and managing emotions branch scores (P = 0.000) than controls. There were no significant differences between groups for the using emotions branch (P = 0.113). In addition, partial correlations controlling for sub-clinical psychopathology in EBP showed the existence of a significant correlation of MSCEIT total score and MSCEIT strategic area score with FAST total score.ConclusionsEBP exhibit deficits in several areas of emotion processing. Performance in emotion processing tasks is associated with social functioning in these patients.Disclosure of interestThe authors have not supplied their declaration of competing interest.

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