A-55 Neuropsychological Findings Associated with Progressive Lesion Development in Cerebral Cavernous Malformation Secondary to PDCD10 Mutation

2021 ◽  
Vol 36 (6) ◽  
pp. 1097-1097
Author(s):  
Matthew Phillips ◽  
Amanda Wisinger ◽  
Joseph Fink

Abstract Objective A rare mutation in the PDCD10 gene causes cerebral cavernous malformations, which can result in multiple brain hemorrhages and hundreds of lesions. Up to 50% of individuals with the PDCD10 mutation remain symptom-free throughout their lives; however, others can suffer from seizures or focal neurologic deficits. Participant: A 67-year-old Caucasian male presented for a neuropsychological re-evaluation (2020) following a progressive decline in his memory and attention dating back 20 years. A baseline neuropsychological evaluation was obtained 7 years ago. Relevant medical history included a 5 mm left falcine meningioma, epilepsy, obstructive sleep apnea, hypertension, and type II diabetes. Neuroimaging revealed over 250 scattered lesions that had advanced over the past 7 years, particularly in the paramedian pontine reticular formation, left inferior cerebellum, and right middle frontal gyrus. Results His neurocognitive profile was marked by mild impairments in immediate verbal memory. The remainder of his cognitive profile was average. Compared to his 2013 evaluation, he showed only a minimal decline in verbal memory, but he showed slight improvements in the domains of executive functioning, visuospatial abilities, and language. Conclusion Despite the marked progression of lesions documented on current neuroimaging, aside from immediate verbal memory problems the patient’s neurocognitive functioning was relatively well-preserved. He even exhibited minor improvements within several domains. Given the patient’s current neurocognitive status, this case illustrates the importance of comprehensive neuropsychological testing in cases that would appear more severe based on neuroimaging data alone. Relatedly, progression of neuropathological disease burden on neuroimaging is not necessarily accompanied by consistent progression of neuropsychological dysfunction.

2003 ◽  
Vol 96 (3_suppl) ◽  
pp. 1093-1106 ◽  
Author(s):  
Annalena Venneri ◽  
Simon Pestell ◽  
Paolo Nichelli

Evidence from neuropsychological studies has suggested that verbal and visuospatial abilities might be differentially involved in time perception and that, because there is specialised competence, the two brain hemispheres might play different roles in time-keeping mechanisms. Reported are results of three experiments in which the time estimates of normal adults were tested using a prospective paradigm while they were engaged in concurrent secondary tasks requiring visuospatial or verbal memory and attention. Analysis showed no convincing evidence in support of a differential role of either verbal or visuospatial abilities in time estimation. The greatest disruption in time accuracy was detected when participants performed the time estimation tasks concurrently with secondary working memory tasks. These findings emphasize the importance of the involvement of specific cognitive systems rather than cognitive domains in the processing of temporal information.


Author(s):  
Vanessa Puetz ◽  
Thomas Günther ◽  
Berrak Kahraman-Lanzerath ◽  
Beate Herpertz-Dahlmann ◽  
Kerstin Konrad

Objectives: Although clear advances have been achieved in the study of early-onset schizophrenia (EOS), little is known to date about premorbid and prodromal neuropsychological functioning in EOS. Method: Here, we report on a case of an adolescent male with EOS who underwent neuropsychological testing before and after illness onset. Results: Marked cognitive deficits in the domains of attention, set-shifting, and verbal memory were present both pre-onset and during the course of schizophrenia, though only deficits in verbal memory persisted after illness-onset and antipsychotic treatment. Conclusion: The findings of this case study suggest that impairments in the verbal memory domain are particularly prominent symptoms of cognitive impairment in prodromal EOS and persist in the course of the disorder, which further demonstrates the difficult clinical situation of adequate schooling opportunities for adolescent patients with EOS.


2001 ◽  
Vol 7 (5) ◽  
pp. 640-646 ◽  
Author(s):  
SHELLI R. KESLER ◽  
RAMONA O. HOPKINS ◽  
LINDELL K. WEAVER ◽  
DUANE D. BLATTER ◽  
HOLLY EDGE-BOOTH ◽  
...  

Magnetic resonance (MR) images and neuropsychological testing data of 69 carbon monoxide (CO) poisoned patients were prospectively obtained within 1 day of CO poisoning, two weeks and six months. CO patients' Day 1 cross-sectional fornix surface area measurements, corrected for head size by using a fornix-to-brain ratio (FBR), were compared to normal age and gender-matched controls. Additionally, a within-subjects analysis was performed comparing the mean areas between CO patients' Day 1, 2 weeks and 6-month FBR. The FBR was correlated with patients' neuropsychological data. There were no significant differences between CO patients' Day 1 fornix measurements compared to normal control subjects. However, significant atrophic changes in the fornix of CO poisoned patients occurred at two weeks with no progressive atrophy at 6 months. By 6 months, CO patients showed significant decline on tests of verbal memory (when practice effects were taken into account), whereas visual memory, processing speed and attention/concentration did not decline. This study indicates that CO results in brain damage and cognitive impairments in the absence of lesions and other neuroanatomic markers. (JINS, 2001, 7, 640–646.)


2018 ◽  
Vol 52 (1) ◽  
pp. 1800740 ◽  
Author(s):  
Nathan E. Cross ◽  
Negar Memarian ◽  
Shantel L. Duffy ◽  
Casey Paquola ◽  
Haley LaMonica ◽  
...  

This study aimed to investigate associations between obstructive sleep apnoea (OSA) and cortical thickness in older adults with subjective and objective cognitive difficulties, who are considered “at-risk” for dementia.83 middle-aged to older adults (51–88 years) underwent neuropsychological testing, polysomnography assessment of OSA and a structural magnetic resonance imaging brain scan. A principal components analysis was performed on OSA measures. Cortical thickness and subcortical volumes were compared to extracted components of “oxygen desaturation” and “sleep disturbance”.Oxygen desaturation was significantly related to reduced cortical thickness in the bilateral temporal lobes (left: r=−0.44, p<0.001; right: r=−0.39, p=0.003). Conversely, sleep disturbance was associated with increased thickness in the right postcentral gyrus (r=0.48, p<0.001), pericalcarine (r=0.50, p=0.005) and pars opercularis (r=0.46, p=0.009) and increased volume of the hippocampus and amygdala. Decreased thickness in the bilateral temporal regions was associated with reduced verbal encoding (r=0.28, p=0.010).Given the clinical significance of this sample in terms of dementia prevention, these changes in grey matter reveal how OSA might contribute to neurodegenerative processes in older adults.


1997 ◽  
Vol 80 (2) ◽  
pp. 515-520 ◽  
Author(s):  
David S. Kreiner ◽  
Matilda Alvarado ◽  
Shawna R. Shockley

Volunteers for psychological research can differ in important ways from nonvolunteers and from the general population. The memory abilities of a sample of 80 college student research volunteers were measured using the Wechsler Memory Scale–Revised. Compared to general population norms, the sample scored significantly higher on General Memory and Visual Memory compared to those with more than 12 years of education, the sample scored significantly lower on General Memory, Verbal Memory, and Attention but significantly higher on Visual Memory. Although results may vary at different universities, researchers should be cautious in assuming that the memory abilities of samples of college student volunteers accurately represent the population.


2013 ◽  
Vol 25 (8) ◽  
pp. 1325-1333 ◽  
Author(s):  
Margaret C. Sewell ◽  
Xiaodong Luo ◽  
Judith Neugroschl ◽  
Mary Sano

ABSTRACTBackground: Physicians often miss diagnosis of mild cognitive impairment (MCI) or early dementia and screening measures can be insensitive to very mild impairments. Other cognitive assessments may take too much time or be frustrating to seniors. This study examined the ability of an audio-recorded scale, developed in Australia, to detect MCI or mild Alzheimer's disease (AD) and compared cognitive domain-specific performance on the audio-recorded scale to in-person battery and common cognitive screens.Method: Seventy-six patients from the Mount Sinai Alzheimer's Disease Research Center were recruited. Patients were aged 75 years or older, with clinical diagnosis of AD or MCI (n = 51) or normal control (n = 25). Participants underwent in-person neuropsychological testing followed by testing with the audio-recorded cognitive screen (ARCS).Results: ARCS provided better discrimination between normal and impaired elderly individuals than either the Mini-Mental State Examination or the clock drawing test. The in-person battery and ARCS analogous variables were significantly correlated, most in the 0.4 to 0.7 range, including verbal memory, executive function/attention, naming, and verbal fluency. The area under the curve generated from the receiver operating characteristic curves indicated high and equivalent discrimination for ARCS and the in-person battery (0.972 vs. 0.988; p = 0.23).Conclusion: The ARCS demonstrated better discrimination between normal controls and those with mild deficits than typical screening measures. Performance on cognitive domains within the ARCS was well correlated with the in-person battery. Completion of the ARCS was accomplished despite mild difficulty hearing the instructions even in very elderly participants, indicating that it may be a useful measure in primary care settings.


2016 ◽  
Vol 22 (7) ◽  
pp. 765-776 ◽  
Author(s):  
Richard J. Caselli ◽  
Amylou C. Dueck ◽  
Dona E.C. Locke ◽  
Bruce R. Henslin ◽  
Travis A. Johnson ◽  
...  

AbstractObjectives: The aim of this study was to assess the association between personality factors and age-related longitudinal cognitive performance, and explore interactions of stress-proneness with apolipoprotein E (APOE) ɛ4, a prevalent risk factor for Alzheimer’s disease (AD). Methods: A total of 510 neuropsychiatrically healthy residents of Maricopa County recruited through media ads (mean age 57.6±10.6 years; 70% women; mean education 15.8±2.4 years; 213 APOE ɛ4 carriers) had neuropsychological testing every 2 years (mean duration follow-up 9.1±4.4 years), and the complete Neuroticism Extraversion Openness Personality Inventory-Revised. Several tests were administered within each of the following cognitive domains: memory, executive skills, language, visuospatial skills, and general cognition. Primary effects on cognitive trajectories and APOE ɛ4 interactions were ascertained with quadratic models. Results: With personality factors treated as continuous variables, Neuroticism was associated with greater decline, and Conscientiousness associated with reduced decline consistently across tests in memory and executive domains. With personality factors trichotomized, the associations of Neuroticism and Conscientiousness were again highly consistent across tests within memory and to a lesser degree executive domains. While age-related memory decline was greater in APOE ɛ4 carriers as a group than ɛ4 noncarriers, verbal memory decline was mitigated in ɛ4 carriers with higher Conscientiousness, and visuospatial perception and memory decline was mitigated in ɛ4 carriers with higher Openness. Conclusions: Neuroticism and Conscientiousness were associated with changes in longitudinal performances on tests sensitive to memory and executive skills. APOE interactions were less consistent. Our findings are consistent with previous studies that have suggested that personality factors, particularly Neuroticism and Conscientiousness are associated with cognitive aging patterns. (JINS, 2016, 22, 765–776)


2004 ◽  
Vol 6 (1) ◽  
pp. 9-22 ◽  
Author(s):  
Stephen M. Rao

Cognitive impairment is common in multiple sclerosis (MS), with up to 65% of patients exhibiting some type of neuropsychological dysfunction. The cognitive domains most affected by MS are learning and memory, attention, information processing, visuospatial abilities, and executive functioning. It is difficult to detect cognitive dysfunction in patients with MS during routine neurologic examinations because conventional measures of neurologic disability are not sensitive enough to detect cognitive impairment. Furthermore, cognitive dysfunction is only weakly correlated with the type of MS, disease duration, or physical disability. However, brain imaging studies show that a relatively strong correlation exists between cognitive dysfunction and overall lesion burden and brain atrophy in MS. This paper reviews the natural history of cognitive dysfunction, areas of cognition affected, the correlation between MRI measures and cognitive dysfunction, issues related to neuropsychological assessment, and treatment of cognitive impairment with disease-modifying MS drugs.


2017 ◽  
Vol 41 (S1) ◽  
pp. S385-S385
Author(s):  
E. Studerus

IntroductionPatients with an at-risk mental state (ARMS) for psychosis and patients with attention deficit hyperactivity disorder (ADHD) have many overlapping symptoms and hence can be difficult to differentiate clinically.ObjectivesThe aim of this study was to investigate whether the differential diagnosis between ARMS and ADHD could be improved by neuropsychological testing.MethodsA total of 157 ARMS and 122 adult ADHD patients were recruited via the Basel Früherkennung von Psychosen (FePsy) study and the ADHD Special Consultations Unit of the University of Basel Psychiatric Hospital, respectively. Verbal learning and memory was tested with the California Verbal Learning Test (CVLT), sustained attention with the Continuous performance test (CPT) and problem solving abilities with the Tower of Hanoi task. Group differences in neuropsychological performance were analyzed using generalized linear models, which included age and gender as covariates.ResultsAdult ADHD patients recalled significantly fewer words in the CVLT (both after short and long delay) and had significantly more false alarms and omissions and longer reaction times in the CPT than ARMS patients.ConclusionsAdult ADHD patients show larger deficits than ARMS patients in the domains of verbal memory and sustained attention, but not in problem solving abilities. This in line with current meta-analyses, which found that impairments in the domains of attention and verbal memory are of medium effect size in adult ADHD patients and of small effect size in ARMS patients. Our results suggest that measures of these domains can be exploited to improve the differential diagnosis between adult ADHD and ARMS patients.Disclosure of interestThe author has not supplied his/her declaration of competing interest.


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