Variant Creutzfeldt-Jakob disease: Neuropsychological profile in an extended series of cases

2009 ◽  
Vol 15 (5) ◽  
pp. 807-810 ◽  
Author(s):  
KARI HAWKINS ◽  
GURJIT CHOHAN ◽  
CHRISTOPHER KIPPS ◽  
ROBERT WILL ◽  
NARINDER KAPUR

AbstractNeuropsychological data on an extended series of cases of variant Creutzfeldt-Jakob Disease (vCJD) are presented, complementing earlier findings from smaller sample studies of this condition. Distinct neuropsychological features in this extended series included relatively preserved verbal knowledge, immediate verbal memory span, and elementary visual processing. This sparing contrasted with ubiquitous impairment in every vCJD patient on timed tests of verbal fluency and digit-symbol substitution. There were also high rates of impairment on tests of memory, and of visuoperceptual and visuospatial reasoning. Our findings lend support to the view that distinctive neuropsychological features may be one of the diagnostic markers of the condition. (JINS, 2009, 15, 807–810.)

2018 ◽  
Vol 32 (1) ◽  
pp. 65-76 ◽  
Author(s):  
Gildas Brébion ◽  
Christian Stephan-Otto ◽  
Susana Ochoa ◽  
Lourdes Nieto ◽  
Montserrat Contel ◽  
...  

2016 ◽  
Vol 119 ◽  
pp. 1-9 ◽  
Author(s):  
Mona Pirmoradi ◽  
Boutheina Jemel ◽  
Anne Gallagher ◽  
Julie Tremblay ◽  
Fabien D’Hondt ◽  
...  
Keyword(s):  

2010 ◽  
Vol 27 (Suppl 1) ◽  
pp. A5.1-A5
Author(s):  
Jacques Kerr

ObjectivesAlthough the majority of head-injured children are Glasgow Coma Scale (GCS) 15 and do not require admission or further investigations there is evidence that some children with a minor head injury (MHI) will go on to experience significant morphological and functional brain deficits. In view of the lack of controlled studies into the sequelae of MHI in children we carried out a pilot study that examined the neuropsychological performance in children who had sustained an MHI.MethodsChildren aged 6–12 years who presented to the Emergency Department of the Royal Hospital for Sick Children in Edinburgh with a MHI (GCS 15) were recruited to the study. Neuropsychological assessment was performed using the Cambridge Neuropsychological Test Automated Battery (CANTAB); a computerised battery of tests, which measures motor skills, visual attention, spatial memory, working memory and non-verbal memory span. CANTAB also assesses executive function, which is the highest level of cognitive function that manages planning and allows flexible thought and action.Results15 head-injured children were recruited over the study period (eleven boys and four girls). A 2 (subject groups) × 3 (time points) repeated measures Analysis of variance was used on the number of problems solved using the minimum number of computer moves at each time point, which showed a significant interaction (p=0.003). Bonferroni posthoc comparisons demonstrated that in week 1 the head-injured group were significantly lower in performance compared to normative data (p=0.03), but significantly increased in performance between week 1 and 12 months (p=0.0001). The difference in the head-injured group performance between week 1 and week 6 approached significance (p=0.05).ConclusionThe MHI group show a significantly poorer performance with regards to problem-solving in a minimum number of moves, which may indicate impulsive tendencies and insufficient planning ability. Children with a MHI may therefore be at risk of developing problems with high-level cognitive function postinjury.


2019 ◽  
Vol 34 (6) ◽  
pp. 925-925
Author(s):  
A Guerra ◽  
J Moses ◽  
J Rivera ◽  
M Davis ◽  
K Hakinson

Abstract Objective Examine whether verbal abilities may help explain the learning strategies people employ when completing a short-term verbal memory task. Methods The assessment records of 296 American Veterans with diverse neuropsychiatric conditions were analyzed using Exploratory Factor Analyses. There were no exclusion criteria. All participants completed the Benton Serial Digit Learning Test – 9 Digits (SDL-9) and Visual Naming (VisNam), Sentence Repetition (SenRep), Controlled Word Association (COWA), and Token Tests of the Multilingual Aphasia Examination (MAE). Individual assessment instruments were factored using Principal Component Analyses (PCA). A three-factor solution of the SDL-9 was co-factored with the verbal components of the MAE to identify common sources of variance. Results A three-factor solution of the SDL-9 separated trials into three overlapping factors consisting of early (SDL-9_Early), middle (SDL-9_Middle), and late (SDL-9_Late) trials. Co-factoring the three new scales with the verbal components of the MAE produced a four-factor model explaining 67.85% of the shared variance: 1) SenRep loaded with SDL-9_Early, 2) COWAT loaded with SDL-9_Middle and SDL-9_Late, 3) Token loaded with SDL-9_Late, and 4) Vis Nam loaded with SDL-9_Late. Conclusions The results suggest that individuals may engage verbal abilities differently as they progress from simpler to more difficult verbal short-term memory tasks. It appears performance in early trials is mostly associated with rote repetition and performance on middle trials is mostly associated with verbal fluency, while performance on the late trials is associated with a combination of verbal fluency, auditory comprehension, and conceptual organization/naming. This may therefore indicate a shift in learning strategy to meet increased cognitive demands.


2006 ◽  
Vol 14 (7S_Part_17) ◽  
pp. P923-P924
Author(s):  
Emilie Thomas ◽  
Tharick A. Pascoal ◽  
Melissa Savard ◽  
Laurie-Anne Dion ◽  
Mira Chamoun ◽  
...  

2013 ◽  
Vol 44 (6) ◽  
pp. 1121-1130 ◽  
Author(s):  
N. Y. Shin ◽  
T. Y. Lee ◽  
E. Kim ◽  
J. S. Kwon

BackgroundSubstantial empirical evidence has indicated impairment in the cognitive functioning of patients with obsessive-compulsive disorder (OCD) despite inconsistencies. Although several confounding factors have been investigated to explain the conflicting results, the findings remain mixed. This study aimed to investigate cognitive dysfunction in patients with OCD using a meta-analytic approach.MethodThe PubMed database was searched between 1980 and October 2012, and reference lists of review papers were examined. A total of 221 studies were identified, of which 88 studies met inclusion criteria. Neuropsychological performance and demographic and clinical variables were extracted from each study.ResultsPatients with OCD were significantly impaired in tasks that measured visuospatial memory, executive function, verbal memory and verbal fluency, whereas auditory attention was preserved in these individuals. The largest effect size was found in the ability to recall complex visual stimuli. Overall effect estimates were in the small to medium ranges for executive function, verbal memory and verbal fluency. The effects of potentially confounding factors including educational level, symptom severity, medication status and co-morbid disorders were not significant.ConclusionsPatients with OCD appear to have wide-ranging cognitive deficits, although their impairment is not so large in general. The different test forms and methods of testing may have influenced the performance of patients with OCD, indicating the need to select carefully the test forms and methods of testing used in future research. The effects of various confounding variables on cognitive functioning need to be investigated further and to be controlled before a definite conclusion can be made.


2020 ◽  
Vol 35 (6) ◽  
pp. 1045-1045
Author(s):  
Leonard S ◽  
Olsen E ◽  
Bradbury K

Abstract Objective Anterior temporal lobectomy (ATL) surgery for epilepsy is commonly associated with post-surgical impairments in memory and language abilities, specifically episodic memory and confrontational naming. Less is known regarding outcomes of patients with atypical language dominance. Method Casey is a 19 year-old, ambidextrous, male with a history of remote symptomatic medically intractable focal epilepsy secondary to right inferior temporal encephalomalacia. Casey underwent right anterior temporal lobectomy and resection of mesial structures including part of the hippocampus. Results Casey’s pre-surgical neuropsychological evaluation revealed overall intellectual functioning in the average range with relative strengths in perceptual reasoning skills and weaknesses in attention, executive control, confrontational naming, and verbal fluency. Casey’s pre-surgical cognitive profile and functional MRI were suggestive of bilateral language organization. At post-surgical evaluation, Casey demonstrated strong overall cognitive abilities, basic reading, visual–spatial skills, and visual memory, as well as notable improvements in his processing speed and visual-motor integration. He demonstrated significant improvement in verbal working memory, verbal fluency, and contextual verbal memory. Casey continued to show significant weaknesses in word finding, phonetic verbal fluency, and rote verbal learning and memory, and milder weaknesses in aspects of executive functioning. Casey continued to demonstrate mood difficulties. Conclusions The current case provides evidence of continued mild deficits in traditional dominant hemisphere skills including rote verbal memory and focal language abilities, in a patient with a history of mixed language dominance, following right hemisphere ATL surgery. This case further highlights the importance of pre-surgical neuropsychological evaluation and fMRI in patients undergoing right ATL surgery.


BMC Neurology ◽  
2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Shu-yang Yu ◽  
Teng-hong Lian ◽  
Peng Guo ◽  
Li-xia Li ◽  
Du-yu Ding ◽  
...  

Abstract Background Apathy is one of the most common symptoms of Alzheimer’s disease (AD), however, correlations of apathy with demographic variables, cognitive functions, neuropsychiatric symptoms, activity of daily living and olfactory functions in AD patients are still lacking comprehensive investigations. Methods This is a cross-sectional study. Total 124 typical AD patients were consecutively recruited from April 2014 to April 2017. In 124 AD patients, 47 cases (37.9%) were male and 77 cases were female; patients’ age were 43–93 years with an average of 68 years. Patients were divided into AD with apathy (AD-A) and AD with no apathy (AD-NA) groups according to the score of Modified Apathy Evaluation Scale, then were evaluated cognitive functions, neuropsychiatric symptoms and activity of daily living, and tested olfactory functions. Above variables were compared between AD-A and AD-NA groups. Further correlation analyses and linear regression analysis were performed between apathy and above variables. Results Compared with AD-NA group, global cognitive level, verbal memory, verbal fluency and activity of daily living were significantly compromised in AD-A group (P < 0.002); depression and agitation were severely displayed in AD-A group (P < 0.002). Apathy was negatively correlated with global cognitive function, verbal memory, verbal fluency and activity of daily living (P < 0.05). There was no significant difference of olfactory functions between the two groups (P > 0.002), and correlations between apathy and olfactory threshold, olfactory identification and global olfactory function were significant (P < 0.05) but quite weak (|r| < 0.3). Further linear regression analysis showed that only verbal fluency and instrumental activities of daily living were independently associated with apathy. Conclusions Independent correlations among apathy, verbal fluency and instrumental activities of daily living in AD patients might be related to the common brain area involved in their pathogeneses.


Neurology ◽  
2016 ◽  
Vol 88 (1) ◽  
pp. 17-24 ◽  
Author(s):  
Justin Centi ◽  
Roy Freeman ◽  
Christopher H. Gibbons ◽  
Sandy Neargarder ◽  
Alexander O. Canova ◽  
...  

Objective:To investigate the relation between orthostatic hypotension (OH) and posture-mediated cognitive impairment in Parkinson disease (PD) using a cross-sectional and within-group design.Methods:Individuals without dementia with idiopathic PD included 18 with OH (PDOH) and 19 without OH; 18 control participants were also included. Neuropsychological tests were conducted in supine and upright-tilted positions. Blood pressure was assessed in each posture.Results:The PD groups performed similarly while supine, demonstrating executive dysfunction in sustained attention and response inhibition, and reduced semantic fluency and verbal memory (encoding and retention). Upright posture exacerbated and broadened these deficits in the PDOH group to include phonemic fluency, psychomotor speed, and auditory working memory. When group-specific supine scores were used as baseline anchors, both PD groups showed cognitive changes following tilt, with the PDOH group exhibiting a wider range of deficits in executive function and memory as well as significant changes in visuospatial function.Conclusions:Cognitive deficits in PD have been widely reported with assessments performed in the supine position, as seen in both our PD groups. Here we demonstrated that those with PDOH had transient, posture-mediated changes in excess of those found in PD without OH. These observed changes suggest an acute, reversible effect. Understanding the effects of OH due to autonomic failure on cognition is desirable, particularly as neuroimaging and clinical assessments collect data only in the supine or seated positions. Identification of a distinct neuropsychological profile in PD with OH has quality of life implications, and OH presents itself as a possible target for intervention in cognitive disturbance.


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