scholarly journals Cognitive Impairment in Adult Patients with 5q-Associated Spinal Muscular Atrophy

2021 ◽  
Vol 11 (9) ◽  
pp. 1184
Author(s):  
Kathrin Kizina ◽  
Yakup Akkaya ◽  
Daniel Jokisch ◽  
Benjamin Stolte ◽  
Andreas Totzeck ◽  
...  

In previous studies, a below-average, average, or above-average intelligence quotient (IQ) in children with SMA was detected but, aside from a severe physical disability, the cognitive performance of adult SMA patients has not yet been evaluated. The intelligence test used in this study, the Wechsler Adult Intelligence Scale, fourth edition (WAIS-IV), was used to measure major intelligence components of adult SMA patients. The WAIS-IV determines four index scores representing verbal comprehension, perceptual reasoning, working memory, and processing speed. Due to time-dependent demands on motor function, the processing speed index score was excluded. IQ index scores of 33 adult SMA patients did not differ from IQ index scores of the normal population. In SMA type-3 patients, the index scores for verbal comprehension, perceptual reasoning, and working memory did not differ from the normal population but showed a trend of IQ scores towards lower points. Patients with SMA type 2 had lower IQ index scores for working memory (90.33 ± 12.95; p = 0.012) and perceptual reasoning (90.73 ± 12.58; p = 0.013) than the normal population. This study provided further evidence that SMA is a multi-systemic disease and may refute the widespread hypothesis that SMA patients might improve their cognitive skills to compensate for their physical impairment.

2021 ◽  
Vol 10 (15) ◽  
pp. 3421
Author(s):  
Steffen Knopke ◽  
Arvid Schubert ◽  
Sophia Marie Häussler ◽  
Stefan Gräbel ◽  
Agnieszka J. Szczepek ◽  
...  

Several studies demonstrated the association of hearing disorders with neurocognitive deficits and dementia disorders, but little is known about the effects of auditory rehabilitation on the cognitive performance of the elderly. Therefore, the research question of the present study was whether cochlear implantation, performed in 21 patients over 70 with bilateral severe hearing impairment, could influence their cognitive skills. The measuring points were before implantation and 12 months after the first cochlear implant (CI) fitting. Evaluation of the working memory (WMI) and processing speed (PSI) was performed using the Wechsler Adult Intelligence Scale 4th edition (WAIS-IV). The audiological assessment included speech perception (SP) in quiet (Freiburg monosyllabic test; FMT), noise (Oldenburg sentence test; OLSA), and self-assessment inventory (Oldenburg Inventory; OI). Twelve months after the first CI fitting, not only the auditory parameters (SP and OI), but also the WMI and PSI, improved significantly (p < 0.05) in the cohort. The presented results imply that cochlear implantation of bilaterally hearing-impaired patients over 70 positively influences their cognitive skills.


2010 ◽  
Vol 106 (1) ◽  
pp. 13-18 ◽  
Author(s):  
Joseph J. Ryan ◽  
Laura A. Glass

The effects of replacing core subtests with supplementary subtests on composite-score reliabilities were evaluated for the WAIS–IV Indexes. Composite score reliabilities and SEMs (i.e., confidence intervals around obtained scores) are provided for the 13 unique Index scores calculated following the subtest substitution guidelines of Wechsler in 2008. In all instances, unique Index composite-score reliabilities were comparable to their respective core Index score composite reliabilities, and measurement error never increased by more than 1 point. Using the standard Verbal Comprehension Index and Perceptual Reasoning Index and the unique subtest combinations for the Working Memory and Processing Speed indexes, which have the lowest composite-score reliabilities, decreased Full Scale composite reliability by .01, while the associated confidence interval of ± 6 represents an increase in measurement error of 1 IQ point.


2018 ◽  
Vol 20 (1) ◽  
pp. 58-67 ◽  
Author(s):  
Lucinda Charlotte Flinn ◽  
Charlotte Louise Hassett ◽  
Louise Braham

Purpose The Wechsler Adult Intelligence Scale – Fourth Edition (WAIS-IV) (Wechsler, 2008) is a cognitive assessment that is often used in secure forensic settings, however it has not been normed on this population. The purpose of this paper is to develop forensic normative data. Design/methodology/approach Patient files in a high secure forensic hospital were reviewed in order to obtain completed WAIS-IV (Wechsler, 2008) assessments and scores from the five indexes (verbal comprehension, perceptual reasoning, working memory, processing speed and full scale intelligence quotient (FSIQ)). This included reviewing patient files from all directorates, including male mental health, male learning disability, male personality disorder and the women’s service, yielding a sample size of n=86. Findings The qualitative descriptors obtained across the hospital ranged between extremely low and superior. The learning disability service scored significantly lower than the mental health and personality disorder services in verbal comprehension index, perceptual reasoning index, working memory index and FSIQ, and significantly lower than the mental health, personality disorder and women’s services in processing speed index. Mean scores from this study were significantly lower in comparison to those from the UK validation study (Wechsler, 2008). Practical implications The significant difference between scores from the current study and those from the UK validation study (Wechsler, 2008) highlights the need to have appropriate normative data for forensic populations. Clinicians should consider interventions that may serve to increase cognitive function, such as cognitive remediation therapy. Originality/value Whilst several special group studies have previously been conducted, this study is the first to develop forensic normative data for the WAIS-IV (Wechsler, 2008). Whilst the sample size was relatively small with limited female participants, the data collated will enable clinicians working in forensic establishments to interpret their assessments in light of this information.


GeroPsych ◽  
2014 ◽  
Vol 27 (3) ◽  
pp. 109-114
Author(s):  
Mitsunobu Kunimi ◽  
Haruyuki Kojima

This study examined the processing speed and memory span of young adults and older people using tasks based on the Wechsler Adult Intelligence Scale-III (WAIS-III, Wechsler, 1997 ). By comparing the data obtained from these tasks, we examined the effects of processing speed and memory span on working memory (WM). In addition, this study examined how presentation modality and the subject’s age are related to WM. Multiple regression analysis of the effect of memory span for each presentation modality used processing time as a factor to predict the WM span of various age groups. The result was two equations for predicting WM span. According to these equations, WM is negatively correlated with “age group” and “processing time,” and positively correlated with “memory span.” Memory span and processing speed were found to have similar impacts on WM, regardless of the presentation modality. However, our results suggested that visual WM and auditory WM are different functions, and that auditory WM is more strongly affected by memory span than visual WM.


Author(s):  
Vishwa CR ◽  
Gargi Das ◽  
Rachna Seth ◽  
Savita Sapra ◽  
Prasanth Siri ◽  
...  

BACKGROUND: Neurocognitive deficits are an important late effect in survivors of acute lymphoblastic Leukemia(ALL). Data from low middle income countries is scarce and highly influenced by biological and cultural variations. Such data would be useful for highlighting the importance of early intervention in an already disadvantaged population. PROCEDURE: 70 consecutive survivors of childhood ALL were evaluated for neurocognitive deficits by the Indian adaptation of Wechsler Intelligence Scale for Children-Fourth Edition(WISC-INDIA). Prevalence of neurocognitive deficits was calculated based on Full Scale Intelligence Quotient(FSIQ) and scores in discrete domains like Verbal Comprehension, Perceptual Reasoning, Working Memory and Processing Speed were calculated and compared to baseline characteristics, chemotherapy and radiation dose received. RESULTS: The mean FSIQ was 86.1 ± 20.5, with significant neurocognitive deficit(FSIQ <90) being prevalent in 50%(95% CI 38% to 62%) of the cohort. The proportion of survivors with deficits in individual domains of verbal comprehension, perceptual reasoning, working memory and processing speed were 49%, 50%, 47% and 44% respectively. The odds of having deficits in neurocognitive function was higher when a child belonged to lower socioeconomic strata, had parents with less than primary school education and whose birth order was higher(All p<0.05). Age at diagnosis, current age at assessment, receiving lower or higher dose of radiotherapy, high dose methotrexate or cytarabine did not have a direct impact on neurocognitive function. CONCLUSIONS AND RELEVANCE: The current need is to develop country specific neurocognition assessment tools to initiate early screening and develop culturally appropriate preventive and rehabilitative interventions.


2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii431-iii431
Author(s):  
Lisa Kahalley ◽  
Rachel Peterson ◽  
M Douglas Ris ◽  
Laura Janzen ◽  
M Fatih Okcu ◽  
...  

Abstract PURPOSE By reducing dose to normal brain tissue, proton radiotherapy (PRT) may lessen neurocognitive risk traditionally associated with photon radiotherapy (XRT). We examined change in neurocognitive scores over time in pediatric medulloblastoma patients treated with PRT versus XRT. METHODS Neurocognitive scores from 79 patients (37 PRT, 42 XRT) were examined. Patients were treated between 2007–2018 on the same treatment protocols that differed only by craniospinal modality (PRT versus XRT). Change in scores over time since diagnosis were compared between groups. RESULTS Groups were similar on most demographic/clinical variables: sex (67.1% male), age at diagnosis (mean 8.6 years), CSI dose (median 23.4 Gy), length of follow-up (mean 4.3 years), and parental education (mean 14.3 years). Boost dose (p&lt;0.001) and margin (p=0.001) differed between groups. Adjusting for covariates, the PRT group exhibited superior outcomes in global IQ, perceptual reasoning, and working memory versus the XRT group (all p&lt;0.05). The XRT group exhibited significant decline in global IQ, working memory, and processing speed (all p&lt;0.05). The PRT group exhibited stable scores in all domains except processing speed (p=0.003). Posterior fossa syndrome imparted risk independent of modality. CONCLUSION This is the first study comparing neurocognitive trajectories between pediatric patients treated for medulloblastoma with PRT versus XRT on comparable, contemporary protocols. PRT was associated with more favorable neurocognitive outcomes in most domains compared to XRT, although processing speed emerged as vulnerable in both groups. This is the strongest evidence to date of an intellectual sparing advantage with PRT in the treatment of pediatric medulloblastoma.


Twin Research ◽  
2003 ◽  
Vol 6 (2) ◽  
pp. 131-139 ◽  
Author(s):  
Daniëlle Posthuma ◽  
Wim F. C. Baaré ◽  
Hilleke E. Hulshoff Pol ◽  
René S. Kahn ◽  
Dorret I. Boomsma ◽  
...  

PLoS ONE ◽  
2021 ◽  
Vol 16 (12) ◽  
pp. e0261208
Author(s):  
Rafael Ferracini Cabral ◽  
Diogo Goulart Corrêa ◽  
Nicolle Zimmermann ◽  
Gustavo Tukamoto ◽  
Tadeu Takao Almodovar Kubo ◽  
...  

Purpose Changes in cerebral cortical regions occur in HIV-infected patients, even in those with mild neurocognitive disorders. Working memory / attention is one of the most affected cognitive domain in these patients, worsening their quality of life. Our objective was to assess whether cortical thickness differs between HIV-infected patients with and without working memory deficit. Methods Forty-one adult HIV-infected patients with and without working memory deficit were imaged on a 1.5 T scanner. Working memory deficit was classified by composite Z scores for performance on the Digits and Letter-Number Sequencing subtests of the Wechsler Adult Intelligence Scale (third edition; WAIS-III). Cortical thickness was determined using FreeSurfer software. Differences in mean cortical thickness between groups, corrected for multiple comparisons using Monte-Carlo simulation, were examined using the query design estimate contrast tool of the FreeSurfer software. Results Greater cortical thickness in left pars opercularis of the inferior frontal gyrus, and rostral and caudal portions of the left middle frontal gyrus (cluster 1; p = .004), and left superior frontal gyrus (cluster 2; p = .004) was observed in HIV-infected patients with working memory deficit compared with those without such deficit. Negative correlations were found between WAIS-III–based Z scores and cortical thickness in the two clusters (cluster 1: ρ = –0.59; cluster 2: ρ = –0.47). Conclusion HIV-infected patients with working memory deficit have regions of greater thickness in the left frontal cortices compared with those without such deficit, which may reflect increased synaptic contacts and/or an inflammatory response related to the damage caused by HIV infection.


2020 ◽  
Vol 46 (Supplement_1) ◽  
pp. S50-S50
Author(s):  
Silvia Amoretti ◽  
Gerard Anmella ◽  
Ana Meseguer ◽  
Cristina Saiz ◽  
Sonia Canals ◽  
...  

Abstract Background The cognitive reserve (CR) refers to the brain’s capacity to cope with pathology in order to minimize the symptoms. In the field of first episode psychosis (FEP), the CR was able to predict functional and neurocognitive performance. Nevertheless, CR has been estimated using heterogeneous methods, which, in term, difficult to compare studies. Therefore, there is a need to create a specific scale for the assessment of this relevant construct. The Cognitive Reserve Assessment Scale in Health (CRASH) is the first measure developed specifically for patients with severe mental illness with optimal psychometric properties, facilitating reliable and valid measurement of CR. The study of the internal structure of the CRASH determined a four-factor structure (Education, Occupation, Leisure activities and Sociability) that can be analyzed separately to know what kind of relationship they might have with other variables. The aim of this study was to analyze the effects of CR measured with CRASH scale on functioning and neurocognitive performance and to explore the relationship of each factor with the outcome in an adult sample of subjects with FEP. Methods The sample of this study came from a multicentre, naturalistic and longitudinal research project financed by a catalan grant (“Pla Estratègic de Recerca i Innovació en Salut” - PERIS 2016–2018). Expedient Nº: SLT006/17/00345; entitled “Identificación y caracterización del valor predictivo de la reserva cognitiva en el curso evolutivo y respuesta en terapéutica en personas con un primer episodio psicótico”. 23 FEP patients and 72 healthy control (HC) were enrolled. The premorbid IQ was estimated with the Wechsler Adult Intelligence Scale (WAIS-IV) vocabulary subtest. To assess processing speed, Trail Making Test-part A was used. Sustained attention was tested with the Continuous Performance Test–II. The working memory was assessed with the Letters and Numbers Subtest of the WAIS-IV. Finally, the executive functions tested set shifting, planning and cognitive flexibility using the Tower of London task and the Trail Making Test (TMT) part B. Results Significant differences between the total CRASH score of patients and HC groups have been found. The patient group obtained lower scores compared to the HC group (36.66±16.01 vs 49.83±11.08, p&lt;0.001). After performing a logistic regression to assess the predictive power of CRASH for each group, the model correctly classified 83.2% of the cases (B=0.091; p&lt;0.001; Exp(B)=1.095). In FEP patients, the CRASH score was associated with premorbid IQ (p&lt;0.001), processing speed (p=0.005), executive function (TMT-B, p=0.005; London Tower task, p=0.039) and attention (CPT Hit SE ISI change, p=0.004). Specifically, the Education factor was associated with premorbid IQ, processing speed, working memory and executive function. The Occupation was only associated with executive function. Leisure activities factor was correlated with premorbid IQ and functioning. Finally, Sociability was correlated with psychosocial functioning and duration of untreated psychosis. In HC, CRASH was associated with premorbid IQ (p&lt;0.001) and attention (p=0.015). Education and Occupation factors were associated with premorbid IQ and attention; Leisure activities with processing speed; and sociability with attention. Discussion FEP patients were shown to have lower CR than HC, and CRASH correctly classified 83.2% of the sample. Each CRASH factor was associated with different outcome, which is why it can be interesting to analyze the total CRASH score and each factor separately. Patients with higher CR showed a better cognitive performance. Therefore, enhancing each factor involved in cognitive reserve may improve outcomes in FEP.


2019 ◽  
Vol 34 (7) ◽  
pp. 1175-1191 ◽  
Author(s):  
Thomas P Ross ◽  
Sydne O’Connor ◽  
Graham Holmes ◽  
Brittany Fuller ◽  
Megan Henrich

Abstract Objective This study examined the test–retest reliability and construct validity of the Action Fluency Test (AFT) as a measure of executive functioning. Method Using a correlational design, 128 healthy college students (M Age = 19.24, SD = 2.01; M education = 13.29 years, SD = 0.81) completed the AFT, and measures of verbal and figural fluency, executive functioning and other relevant constructs (e.g., vocabulary, working memory, and attention). Results Coefficients of stability were acceptable for AFT correct words (r = .76; p &lt; .01), but not for errors (r = .41) or perseverations (r = .14). No practice effects were observed upon repeat testing (M interval = 39.21 days). Divergent validity evidence was mixed. AFT scores were unrelated to working memory and perceptual-reasoning abilities; however, correlations with vocabulary (r = .32; p &lt; .01) and information-processing speed (r = .30; p &lt; .01) were greater than associations between AFT scores and executive measures. Regarding convergent validity, AFT scores correlated with other fluency tasks (r = .4 range), but correlations with measures of executive functioning were absent or small. Action and letter fluency correlated with measures of attentional control and inhibition; however, these associations were no longer significant after controlling for shared variance with information-processing speed. Conclusions Findings are consistent with previous research suggesting vocabulary and information-processing speed underlie effective fluency performance to a greater extent than executive functioning. The AFT measures unique variance not accounted for by semantic and letter fluency tasks, and therefore may be used for a variety of research and clinical purposes.


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