Cognitive and Behavioral Neurology最新文献

{"title":"Memory Loss, Alzheimer's Disease, and Dementia: A Practical Guide for Clinicians, 3rd ed.","authors":"Howard S Kirshner","doi":"10.1097/WNN.0000000000000323","DOIUrl":"https://doi.org/10.1097/WNN.0000000000000323","url":null,"abstract":"","PeriodicalId":50671,"journal":{"name":"Cognitive and Behavioral Neurology","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2022-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41154673","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Swimmers: A Novel.","authors":"Howard S Kirshner","doi":"10.1097/WNN.0000000000000315","DOIUrl":"https://doi.org/10.1097/WNN.0000000000000315","url":null,"abstract":"","PeriodicalId":50671,"journal":{"name":"Cognitive and Behavioral Neurology","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2022-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40343741","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Validation of a Mobile, Sensor-based Neurobehavioral Assessment With Digital Signal Processing and Machine-learning Analytics.","authors":"Kelly L Sloane,&nbsp;Joel A Mefford,&nbsp;Zilong Zhao,&nbsp;Man Xu,&nbsp;Guifeng Zhou,&nbsp;Rachel Fabian,&nbsp;Amy E Wright,&nbsp;Shenly Glenn","doi":"10.1097/WNN.0000000000000308","DOIUrl":"10.1097/WNN.0000000000000308","url":null,"abstract":"<p><strong>Background: </strong>The Miro Health Mobile Assessment Platform consists of self-administered neurobehavioral and cognitive assessments that measure behaviors typically measured by specialized clinicians.</p><p><strong>Objective: </strong>To evaluate the Miro Health Mobile Assessment Platform's concurrent validity, test-retest reliability, and mild cognitive impairment (MCI) classification performance.</p><p><strong>Method: </strong>Sixty study participants were evaluated with Miro Health version V.2. Healthy controls (HC), amnestic MCI (aMCI), and nonamnestic MCI (naMCI) ages 64-85 were evaluated with version V.3. Additional participants were recruited at Johns Hopkins Hospital to represent clinic patients, with wider ranges of age and diagnosis. In all, 90 HC, 21 aMCI, 17 naMCI, and 15 other cases were evaluated with V.3. Concurrent validity of the Miro Health variables and legacy neuropsychological test scores was assessed with Spearman correlations. Reliability was quantified with the scores' intraclass correlations. A machine-learning algorithm combined Miro Health variable scores into a Risk score to differentiate HC from MCI or MCI subtypes.</p><p><strong>Results: </strong>In HC, correlations of Miro Health variables with legacy test scores ranged 0.27-0.68. Test-retest reliabilities ranged 0.25-0.79, with minimal learning effects. The Risk score differentiated individuals with aMCI from HC with an area under the receiver operator curve (AUROC) of 0.97; naMCI from HC with an AUROC of 0.80; combined MCI from HC with an AUROC of 0.89; and aMCI from naMCI with an AUROC of 0.83.</p><p><strong>Conclusion: </strong>The Miro Health Mobile Assessment Platform provides valid and reliable assessment of neurobehavioral and cognitive status, effectively distinguishes between HC and MCI, and differentiates aMCI from naMCI.</p>","PeriodicalId":50671,"journal":{"name":"Cognitive and Behavioral Neurology","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2022-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40397046","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Graph Theory Analysis of Semantic Fluency in Russian-English Bilinguals.","authors":"Vidushi Sinha, Frances Lissemore, Alan J Lerner","doi":"10.1097/WNN.0000000000000312","DOIUrl":"10.1097/WNN.0000000000000312","url":null,"abstract":"<p><strong>Background: </strong>Semantic category fluency is a widely used task involving language, memory, and executive function. Previous studies of bilingual semantic fluency have shown only small differences between languages. Graph theory analyzes complex relationships in networks, including node and edge number, clustering coefficient, average path length, average number of direct neighbors, and scale-free and small-world properties.</p><p><strong>Objective: </strong>To shed light on whether the underlying neural processes involved in semantic category fluency testing yield substantially different networks in different languages.</p><p><strong>Method: </strong>We compared languages and methods using both network analysis and conventional analysis of word production. We administered the animal naming task to 51 Russian-English bilinguals in each language. We constructed network graphs using three methods: (a) simple association of unique co-occurring neighbors, (b) corrected associations between consecutive words occurring beyond chance, and (c) a network community approach using planar maximally filtered graphs. We compared the resultant network analytics as well as their scale-free and small-world properties.</p><p><strong>Results: </strong>Participants produced more words in Russian than in English. Small-worldness metrics were variable between Russian and English but were consistent across the three graph theory analytical methods.</p><p><strong>Conclusion: </strong>The networks had similar graph theory properties in both languages. The optimal methodology for creating networks from semantic category fluency remains to be determined.</p>","PeriodicalId":50671,"journal":{"name":"Cognitive and Behavioral Neurology","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2022-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10154949/pdf/nihms-1814366.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10130340","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Phenotypic and Brain Imaging Findings Associated With a 10p Proximal Deletion Including the WAC Gene: Case Report and Literature Review.","authors":"Hilmi Bolat,&nbsp;Hatice Derin,&nbsp;Gül Ünsel-Bolat","doi":"10.1097/WNN.0000000000000309","DOIUrl":"10.1097/WNN.0000000000000309","url":null,"abstract":"<p><p>Microarray-based techniques are an important testing method in etiological studies of intellectual disability and autism spectrum disorder. Interstitial deletion in the p11-p12 region of chromosome 10 is rare, having been reported in just 12 cases to date. Intellectual disability associated with the WAC gene in this region is referred to as DeSanto-Shinawi syndrome . Although all individuals with p11-p12 region of chromosome 10 deletion share a common phenotype involving intellectual disability and dysmorphic features, individuals with DeSanto-Shinawi syndrome usually do not experience the cardiac and neurologic abnormalities or cryptorchidism associated with a 10p11-p12 deletion. With this case report, we aim to expand the phenotypic spectrum of 10p11-p12 deletion. Our patient was a 9-year-old boy with intellectual disability, autism symptoms, dysmorphic features, and behavioral abnormalities. He had no cardiac problems or neurologic symptoms such as hypotonia, feeding difficulties, or seizures. However, he presented cryptorchidism in addition to symptoms that are consistent with DeSanto-Shinawi syndrome. Array comparative genomic hybridization of genomic DNA isolated from a peripheral blood sample revealed a heterozygous deletion in 10p11.23-p12.1, which contains the WAC gene. We discuss our case in the context of a literature review of candidate genes. It is still difficult to establish genotype-phenotype correlations for neurologic, cardiac, and visual symptoms, and cryptorchidism, in individuals with a 10p11-p12 deletion. As more individuals are diagnosed with deletion in this chromosomal region, the associated phenotypes will become clearer.</p>","PeriodicalId":50671,"journal":{"name":"Cognitive and Behavioral Neurology","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2022-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40405609","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Distinguishing Between Genuine and Feigned Dementia Using Event-related Potentials.","authors":"August M Price,&nbsp;Rocco Palumbo,&nbsp;Anna Marin,&nbsp;Prayerna Uppal,&nbsp;Cheongmin Suh,&nbsp;Andrew E Budson,&nbsp;Katherine W Turk","doi":"10.1097/WNN.0000000000000311","DOIUrl":"10.1097/WNN.0000000000000311","url":null,"abstract":"<p><strong>Background: </strong>Individuals with probable Alzheimer disease (AD) may perform below cutoffs on traditional, memory-based performance validity tests. Previous studies have found success using event-related potentials (ERPs) to detect feigned neurocognitive impairment in younger populations.</p><p><strong>Objective: </strong>To evaluate the utility of an auditory oddball task in conjunction with the P3b peak amplitude to distinguish probable AD from simulated dementia.</p><p><strong>Method: </strong>Twenty individuals with probable AD and 20 older healthy controls (HC) underwent an ERP auditory oddball protocol and the Test of Memory Malingering (TOMM). The HC were asked to perform honestly for one condition and to simulate dementia for the other. The individuals with probable AD were asked to perform honestly. The P3b peak amplitude and button press accuracy were collected from each participant and were analyzed to determine their effectiveness in detecting performance validity.</p><p><strong>Results: </strong>The P3b peak amplitude remained stable regardless of behavioral condition in the HC group. When combined with the TOMM Trial 2 score, the P3b peak amplitude further improved the ability to correctly differentiate individuals with probable AD from HC simulating dementia with 100% sensitivity and 90% specificity.</p><p><strong>Conclusion: </strong>The P3b peak amplitude was found to be an effective physiologic measure of cognitive impairment in individuals with probable AD compared with HC simulating dementia. When combined with the TOMM Trial 2 score, the P3b peak amplitude served as a promising performance validity measure for differentiating individuals with probable AD from HC simulating dementia.</p>","PeriodicalId":50671,"journal":{"name":"Cognitive and Behavioral Neurology","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2022-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9444996/pdf/nihms-1813003.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10505581","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Executive Dysfunction, Social Cognition Impairment, and Gray Matter Pathology in Myotonic Dystrophy Type 2: A Pilot Study.","authors":"Thomas Theodosiou,&nbsp;Foteini Christidi,&nbsp;Sofia Xirou,&nbsp;Efstratios Karavasilis,&nbsp;Peter Bede,&nbsp;Constantinos Papadopoulos,&nbsp;Georgios D Argyropoulos,&nbsp;Panagiotis Kourtesis,&nbsp;Varvara Pantolewn,&nbsp;Panagiotis Ferentinos,&nbsp;Evangelia Kararizou,&nbsp;Georgios Velonakis,&nbsp;Ioannis Zalonis,&nbsp;Georgios Papadimas","doi":"10.1097/WNN.0000000000000314","DOIUrl":"10.1097/WNN.0000000000000314","url":null,"abstract":"<p><strong>Background: </strong>In contrast to myotonic dystrophy type 1, the cognitive and radiologic profile of myotonic dystrophy type 2 (DM2) is relatively poorly characterized.</p><p><strong>Objective: </strong>To conduct a pilot study to systematically evaluate cognitive and radiologic features in a cohort of Greek individuals with DM2.</p><p><strong>Method: </strong>Eleven genetically confirmed individuals with DM2 and 26 age- and education-matched healthy controls were administered the Edinburgh Cognitive and Behavioural Amyotrophic Lateral Sclerosis Screen (ECAS) to screen for impairment in multiple cognitive domains. MRI data were evaluated by morphometric analyses to identify disease-specific gray and white matter alterations. The following statistical thresholds were used for cognitive comparisons: PFDR < 0.05 and Bayes factor (BF 10 ) >10.</p><p><strong>Results: </strong>The DM2 group exhibited cognitive impairment (ECAS Total score; PFDR = 0.001; BF 10 = 108.887), which was dominated by executive impairment ( PFDR = 0.003; BF 10 = 25.330). A trend toward verbal fluency impairment was also identified. No significant impairments in memory, language, or visuospatial function were captured. The analysis of subscores revealed severe impairments in social cognition and alternation. Voxel-based morphometry identified widespread frontal, occipital, and subcortical gray matter atrophy, including the left superior medial frontal gyrus, right medial orbitofrontal gyrus, right operculum, right precuneus, bilateral fusiform gyri, and bilateral thalami.</p><p><strong>Conclusion: </strong>DM2 may be associated with multifocal cortical and thalamic atrophy, which is likely to underpin the range of cognitive manifestations mostly characterized by executive impairment and specifically by impaired social cognition.</p>","PeriodicalId":50671,"journal":{"name":"Cognitive and Behavioral Neurology","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2022-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40617366","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Nonparaneoplastic Anti-GAD Limbic Encephalitis: Seizure Outcome and Long-term Neuropsychological Follow-up After Immunotherapy.","authors":"Martha Spilioti,&nbsp;Andreas Kiryttopoulos,&nbsp;Theodora Panou,&nbsp;Panagiotis Simos,&nbsp;Haris Alexopoulos,&nbsp;Georgios Karafyles,&nbsp;Triantafillos Geroukis,&nbsp;Ioannis Kalevrosoglou,&nbsp;Vasilios Kimiskidis","doi":"10.1097/WNN.0000000000000313","DOIUrl":"10.1097/WNN.0000000000000313","url":null,"abstract":"<p><p>Antibodies against glutamate decarboxylase (GAD-Abs), especially GAD65 antibodies, are associated with limbic encephalitis (LE) manifested by temporal lobe epilepsy and neuropsychological deficits. We present the case of a 42-year-old Greek woman with nonparaneoplastic anti-GAD LE, discussing the therapeutic management and highlighting the role of neuropsychological assessment. The patient underwent functional and structural brain studies and was investigated longitudinally over a 6-year period with a battery of neuropsychological tests that were designed to document her intellectual function and verbal and visual memory. The patient suffered from refractory temporal-impaired awareness seizures and memory impairment that was mediated by autoimmune nonparaneoplastic LE and comorbid autoimmune disorders (ie, Hashimoto thyroiditis and vitiligo). Neuroimaging studies demonstrated hyperintensities in the medial temporal lobes bilaterally on T2WI MRI sequences. Serial EEGs showed bitemporal intermittent delta activity as well as epileptiform discharges. Tumor blood markers and onconeural antibodies were negative. Immunological screening revealed extremely high GAD-Abs titers in both serum and CSF, as well as the presence of CSF oligoclonal bands. Neuropsychological testing revealed anterograde amnesia with relative preservation of more remote, premorbid memories. The patient underwent first-line immunotherapy followed by immunosuppressive maintenance treatment that led to a reduction of seizures, EEG improvement, and a significant decline in GAD-Abs titers. Neuropsychological evaluations at 5 months, 1 year, and 6 years posttreatment demonstrated improvement, particularly in recent memory and everyday functionality. In this case of anti-GAD LE, the long-term seizure reduction and the improvement of neuropsychological deficits were most likely related to the immunotherapy.</p>","PeriodicalId":50671,"journal":{"name":"Cognitive and Behavioral Neurology","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2022-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40521587","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"BNT-15: Revised Performance Validity Cutoffs and Proposed Clinical Classification Ranges.","authors":"Kaitlyn Abeare,&nbsp;Laura Cutler,&nbsp;Kelly Y An,&nbsp;Parveen Razvi,&nbsp;Matthew Holcomb,&nbsp;Laszlo A Erdodi","doi":"10.1097/WNN.0000000000000304","DOIUrl":"https://doi.org/10.1097/WNN.0000000000000304","url":null,"abstract":"<p><strong>Background: </strong>Abbreviated neurocognitive tests offer a practical alternative to full-length versions but often lack clear interpretive guidelines, thereby limiting their clinical utility.</p><p><strong>Objective: </strong>To replicate validity cutoffs for the Boston Naming Test-Short Form (BNT-15) and to introduce a clinical classification system for the BNT-15 as a measure of object-naming skills.</p><p><strong>Method: </strong>We collected data from 43 university students and 46 clinical patients. Classification accuracy was computed against psychometrically defined criterion groups. Clinical classification ranges were developed using a z -score transformation.</p><p><strong>Results: </strong>Previously suggested validity cutoffs (≤11 and ≤12) produced comparable classification accuracy among the university students. However, a more conservative cutoff (≤10) was needed with the clinical patients to contain the false-positive rate (0.20-0.38 sensitivity at 0.92-0.96 specificity). As a measure of cognitive ability, a perfect BNT-15 score suggests above average performance; ≤11 suggests clinically significant deficits. Demographically adjusted prorated BNT-15 T-scores correlated strongly (0.86) with the newly developed z -scores.</p><p><strong>Conclusion: </strong>Given its brevity (<5 minutes), ease of administration and scoring, the BNT-15 can function as a useful and cost-effective screening measure for both object-naming/English proficiency and performance validity. The proposed clinical classification ranges provide useful guidelines for practitioners.</p>","PeriodicalId":50671,"journal":{"name":"Cognitive and Behavioral Neurology","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2022-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41118587","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Repeatable Battery for the Assessment of Neuropsychological Status, While Useful for Measuring Cognitive Changes in Manifest Huntington Disease, May Show Limited Utility in Premanifest Disease.","authors":"Andrea I Mustafa,&nbsp;Jody Corey-Bloom,&nbsp;Ilex Beltran-Najera,&nbsp;Chase Snell,&nbsp;Jordan Castleton,&nbsp;Haileigh Smith,&nbsp;Paul E Gilbert","doi":"10.1097/WNN.0000000000000310","DOIUrl":"10.1097/WNN.0000000000000310","url":null,"abstract":"<p><strong>Background: </strong>The Repeatable Battery for the Assessment of Neuropsychological Status (RBANS) is a brief, standardized neuropsychological test that assesses several areas of cognitive function. Recent studies, although sparse, have examined the use of the RBANS to detect cognitive deficits in individuals with manifest Huntington disease (HD); however, no studies have investigated its utility to detect cognitive deficits in individuals with premanifest HD (PreHD), where cognitive symptoms are thought to be more subtle.</p><p><strong>Objective: </strong>To assess cognitive deficits in individuals with HD, particularly in individuals with PreHD, using an easily administered, brief but comprehensive, neuropsychological test.</p><p><strong>Method: </strong>We administered the RBANS to 31 individuals with HD, 29 individuals with PreHD, and 22 healthy controls (HC) at an academic HD clinical research center and collected RBANS Total, Index, and subtest scores for group comparisons.</p><p><strong>Results: </strong>The HD group had significantly lower RBANS Total, Index, and subtest scores than the HC. The PreHD group had significantly lower RBANS Total scores and Coding subtest scores than the HC, but no other significant group differences were identified.</p><p><strong>Conclusion: </strong>Our results substantiate previous findings of significant impairment on the RBANS in individuals with HD. In addition, we are the first to demonstrate that, although the RBANS can identify deficits in psychomotor speed and information processing in individuals with PreHD, it does not appear to have the ability to detect impairment in any additional cognitive domains in individuals with PreHD.</p>","PeriodicalId":50671,"journal":{"name":"Cognitive and Behavioral Neurology","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2022-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40600292","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}