Cognitive and Behavioral Neurology最新文献

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Dynamic Aphasia as a Variant of Frontotemporal Dementia. 动态失语症是额颞叶痴呆的一种变体。
IF 1.4 4区 医学
Cognitive and Behavioral Neurology Pub Date : 2021-12-02 DOI: 10.1097/WNN.0000000000000289
Adithya Chandregowda, Heather M Clark, Joseph R Duffy, Mary M Machulda, Val J Lowe, Jennifer L Whitwell, Keith A Josephs
{"title":"Dynamic Aphasia as a Variant of Frontotemporal Dementia.","authors":"Adithya Chandregowda,&nbsp;Heather M Clark,&nbsp;Joseph R Duffy,&nbsp;Mary M Machulda,&nbsp;Val J Lowe,&nbsp;Jennifer L Whitwell,&nbsp;Keith A Josephs","doi":"10.1097/WNN.0000000000000289","DOIUrl":"10.1097/WNN.0000000000000289","url":null,"abstract":"<p><p>We describe two individuals with progressive verbal difficulty who exhibited impairment of propositional language, with relatively well-preserved auditory comprehension, naming, and repetition-a profile that is consistent with dynamic aphasia. By providing a brief review of pertinent literature and the results from our neurologic, speech and language, neuropsychological, and neuroimaging testing, this report sheds light on the infrequently reported dynamic aphasia in the context of frontotemporal dementia. Our patients' insights into their verbal difficulty tend to support the notion that dynamic aphasia results from interference at the stage where thoughts are converted into verbal messages-that is, the thought-verbal interface.</p>","PeriodicalId":50671,"journal":{"name":"Cognitive and Behavioral Neurology","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2021-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8647805/pdf/nihms-1742999.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39682744","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}
引用次数: 1
Impact of Donepezil and Memantine on Behavioral and Psychological Symptoms of Alzheimer Disease: Six-month Open-label Study. 多奈哌齐和美金刚对阿尔茨海默病行为和心理症状的影响:为期六个月的开放标签研究。
IF 1.4 4区 医学
Cognitive and Behavioral Neurology Pub Date : 2021-12-02 DOI: 10.1097/WNN.0000000000000285
Petra Bago Rožanković, Marjan Rožanković, Jasna Badžak, Maristela Stojić, Ivana Šušak Sporiš
{"title":"Impact of Donepezil and Memantine on Behavioral and Psychological Symptoms of Alzheimer Disease: Six-month Open-label Study.","authors":"Petra Bago Rožanković,&nbsp;Marjan Rožanković,&nbsp;Jasna Badžak,&nbsp;Maristela Stojić,&nbsp;Ivana Šušak Sporiš","doi":"10.1097/WNN.0000000000000285","DOIUrl":"10.1097/WNN.0000000000000285","url":null,"abstract":"Background: Behavioral and psychological symptoms of dementia (BPSD) are common in individuals with Alzheimer disease (AD). Donepezil and memantine are both widely used for the treatment of moderate AD. Objective: To evaluate the effects of donepezil and memantine in relieving BPSD in individuals with moderate AD. Method: We conducted a prospective, randomized, 6-month clinical trial involving 85 individuals with moderate AD divided into two groups: group 1 (n = 42) was treated with donepezil; group 2 (n = 43) was treated with memantine. We used the Neuropsychiatric Inventory (NPI) to assess the prevalence and severity of BPSD at baseline and after 6 months of treatment with donepezil or memantine. Results: The two groups’ baseline characteristics, including age, sex, mean length of education, and disease duration, were comparable, as were their baseline Mini-Mental State Examination scores. The NPI Total score improved from baseline to month 6 in both groups (P < 0.0001). Analyses of the NPI subdomains revealed that both donepezil treatment and memantine treatment produced statistically significant improvement in all of the NPI domains except euphoria and apathy, for which no improvement was observed after memantine treatment. Both treatments were well tolerated, with mostly mild and transient adverse effects. Conclusion: Specific drugs for AD, including donepezil and memantine, may be effective in treating BPSD in individuals with moderate AD, with a favorable safety profile.","PeriodicalId":50671,"journal":{"name":"Cognitive and Behavioral Neurology","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2021-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39682742","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}
引用次数: 5
Intellectual Curiosity and Action Initiation are Subtypes of Apathy Affected in Huntington Disease Gene Expansion Carriers. 智力好奇心和行动启动是亨廷顿舞蹈症基因扩增携带者中受影响的Apathy的亚型。
IF 1.4 4区 医学
Cognitive and Behavioral Neurology Pub Date : 2021-12-02 DOI: 10.1097/WNN.0000000000000286
Rebecca K Hendel, Marie N N Hellem, Lena E Hjermind, Jørgen E Nielsen, Asmus Vogel
{"title":"Intellectual Curiosity and Action Initiation are Subtypes of Apathy Affected in Huntington Disease Gene Expansion Carriers.","authors":"Rebecca K Hendel,&nbsp;Marie N N Hellem,&nbsp;Lena E Hjermind,&nbsp;Jørgen E Nielsen,&nbsp;Asmus Vogel","doi":"10.1097/WNN.0000000000000286","DOIUrl":"10.1097/WNN.0000000000000286","url":null,"abstract":"<p><strong>Background: </strong>Apathy is a prevalent behavioral syndrome of Huntington disease (HD) that can result in severe loss of function for the individual with HD and substantial caregiver distress. Research-based evidence of apathy is characterized by methodological differences, and there is a deficiency in the evidence concerning the subtypes of apathy.</p><p><strong>Objective: </strong>To characterize apathy in premanifest and motor-manifest HD gene expansion carriers and controls using the Short Problem Behaviors Assessment for Huntington's Disease (PBA-s) and the Lille Apathy Rating Scale (LARS).</p><p><strong>Method: </strong>We included 82 HD gene expansion carriers (premanifest and motor manifest) and 32 controls (Mini-Mental State Examination score ≥24 and Montreal Cognitive Assessment score ≥19) in the study. We quantified apathy using the PBA-s and the LARS and performed correlation analyses between the global LARS score and motor function, cytosine-adenine-guanine repeat length, cytosine-adenine-guanine Age Product score, and neuropsychiatric and cognitive symptoms.</p><p><strong>Results: </strong>The motor-manifest HD gene expansion carriers scored significantly higher than the controls on the global score and the Intellectual Curiosity and Action Initiation subscales of the LARS. Apathy was present in 28% of the HD gene expansion carriers (including 7 premanifest). The apathetic participants had a significantly higher motor score, significantly higher scores on the neuropsychiatric instruments, and significantly lower cognitive scores compared with the controls.</p><p><strong>Conclusion: </strong>Apathy is a frequent syndrome that is found in individuals with HD. Apathy has a specific expression, with symptoms such as reduced initiation, voluntary actions, and interests, that might be related to the underlying neuropathology. Apathy is related to disease progression, neuropsychiatric symptoms, and cognitive impairments.</p>","PeriodicalId":50671,"journal":{"name":"Cognitive and Behavioral Neurology","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2021-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39682743","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}
引用次数: 2
Honorific Speech Impairment: A Characteristic Sign of Frontotemporal Dementia. 敬语障碍:额颞叶痴呆的一个特征性体征。
IF 1.4 4区 医学
Cognitive and Behavioral Neurology Pub Date : 2021-12-02 DOI: 10.1097/WNN.0000000000000284
Weon Kyeong Hong, Ji Hye Yoon, Hyemin Jang, Soo Jin Yoon, So Young Moon, Hee Jin Kim, Duk L Na
{"title":"Honorific Speech Impairment: A Characteristic Sign of Frontotemporal Dementia.","authors":"Weon Kyeong Hong,&nbsp;Ji Hye Yoon,&nbsp;Hyemin Jang,&nbsp;Soo Jin Yoon,&nbsp;So Young Moon,&nbsp;Hee Jin Kim,&nbsp;Duk L Na","doi":"10.1097/WNN.0000000000000284","DOIUrl":"10.1097/WNN.0000000000000284","url":null,"abstract":"<p><strong>Background: </strong>Individuals with the behavioral variant of frontotemporal dementia (bvFTD) exhibit various levels of abulia, disinhibition, impaired judgment, and decline in executive function. Empirical evidence has shown that individuals with bvFTD also often exhibit difficulty using honorific speech, which expresses respect to another party or addressee.</p><p><strong>Objective: </strong>To analyze differences in the ability to use honorific speech among individuals with bvFTD, individuals with dementia of the Alzheimer type (AD dementia), and individuals with normal cognition (NC).</p><p><strong>Method: </strong>A total of 53 native Korean speakers (13 bvFTD, 20 AD dementia, and 20 NC) completed an experimental honorific speech task (HST) that involved both expressive and receptive tasks. We analyzed the number of correct responses and error patterns separately for an expressive task and for a receptive task.</p><p><strong>Results: </strong>The bvFTD group had significantly fewer correct responses on the HST compared with the AD dementia and NC groups. The bvFTD group exhibited more misjudgment errors in identifying nonhonorific speech as honorific speech in the expressive task, and significantly longer response times in the receptive task, than the AD dementia and NC groups. Significant associations were identified between HST scores and cortical atrophy in the temporal and frontotemporal lobes.</p><p><strong>Conclusion: </strong>A decline in the ability to use honorific speech may be a diagnosable behavioral and psychiatric symptom for bvFTD in Korean-speaking individuals. This decline in individuals with bvFTD could be attributed to multiple factors, including social manners (politeness) and impaired social language use ability (pragmatics).</p>","PeriodicalId":50671,"journal":{"name":"Cognitive and Behavioral Neurology","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2021-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39682741","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}
引用次数: 0
Disconnected Motor Intention and Spatial Attention in a Case of Probable Marchiafava-Bignami Disease. 一例疑似马尔基亚法瓦-比格纳米病患者的运动意向与空间注意的脱节。
IF 1.4 4区 医学
Cognitive and Behavioral Neurology Pub Date : 2021-09-02 DOI: 10.1097/WNN.0000000000000274
Shunsuke Kobayashi, Masaki Hirose, Yukiko Akutsu, Kazumi Hirayama, Yoshinori Ishida, Yoshikazu Ugawa
{"title":"Disconnected Motor Intention and Spatial Attention in a Case of Probable Marchiafava-Bignami Disease.","authors":"Shunsuke Kobayashi,&nbsp;Masaki Hirose,&nbsp;Yukiko Akutsu,&nbsp;Kazumi Hirayama,&nbsp;Yoshinori Ishida,&nbsp;Yoshikazu Ugawa","doi":"10.1097/WNN.0000000000000274","DOIUrl":"10.1097/WNN.0000000000000274","url":null,"abstract":"<p><p>Marchiafava-Bignami disease (MBD) is a rare complication of chronic alcoholism that typically causes demyelination and necrosis of the corpus callosum. Here, we report a man with probable MBD with callosal and right medial paracentral lesions who presented with abnormal reaching behavior and ideomotor apraxia of the left hand. He exhibited difficulty in reaching with the left hand when a target object was placed on his right-hand side, and he exhibited rightward bias when using his right hand in a line bisection task. These disturbances in reaching suggest disruption of the top-down control of motor intention and spatial attention at the corpus callosum.</p>","PeriodicalId":50671,"journal":{"name":"Cognitive and Behavioral Neurology","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2021-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39378552","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}
引用次数: 1
Brain F-18 FDG and F-18 FP-CIT PET/CT Findings of c.856_860delCTCTA Mutation McLeod Syndrome. c.856_860delCTCTA突变McLeod综合征的脑F-18FDG和F-18FP-CIT PET/CT表现。
IF 1.4 4区 医学
Cognitive and Behavioral Neurology Pub Date : 2021-09-02 DOI: 10.1097/WNN.0000000000000267
Ho-Sung Ryu, Chae Moon Hong
{"title":"Brain F-18 FDG and F-18 FP-CIT PET/CT Findings of c.856_860delCTCTA Mutation McLeod Syndrome.","authors":"Ho-Sung Ryu,&nbsp;Chae Moon Hong","doi":"10.1097/WNN.0000000000000267","DOIUrl":"10.1097/WNN.0000000000000267","url":null,"abstract":"<p><p>McLeod syndrome is a rare X-linked recessive genetic disorder that is caused by mutations of the XK gene. It is one of the core neuroacanthocytosis syndromes. We report the case of a 67-year-old man who presented to Kyungpook National University Hospital in the Republic of Korea with progressive worsening of generalized chorea and dystonia. He had no recognized family history of neurologic illness. A peripheral blood smear showed increased acanthocytes. His serum creatine kinase levels were 894 U/L. A brain MRI showed atrophy of the bilateral striatal nuclei. An F-18 F-N-(3-fluoropropyl)-2β-carboxymethoxy-3β-(4-iodophenyl) nortropane PET/CT showed moderately decreased dopamine transporter uptake in the putamen and severely decreased uptake in the caudate nucleus. An F-18 fludeoxyglucose PET/CT demonstrated markedly decreased metabolism at the caudate nucleus and the putamen. Whole exome sequencing revealed hemizygous pathogenic mutations of the XK gene (c.856_860delCTCTA;p.Leu286TyrfsTer16). We believe that these findings provide useful information regarding the clinical features of individuals with McLeod syndrome.</p>","PeriodicalId":50671,"journal":{"name":"Cognitive and Behavioral Neurology","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2021-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39394199","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}
引用次数: 1
Prevalence of Sleep Disorders and Related Factors in Individuals Undergoing Hemodialysis. 血液透析患者睡眠障碍的患病率及相关因素
IF 1.4 4区 医学
Cognitive and Behavioral Neurology Pub Date : 2021-09-02 DOI: 10.1097/WNN.0000000000000278
Seher Kir, İdris Kirhan, Melda Dilek
{"title":"Prevalence of Sleep Disorders and Related Factors in Individuals Undergoing Hemodialysis.","authors":"Seher Kir,&nbsp;İdris Kirhan,&nbsp;Melda Dilek","doi":"10.1097/WNN.0000000000000278","DOIUrl":"https://doi.org/10.1097/WNN.0000000000000278","url":null,"abstract":"<p><strong>Background: </strong>The quality of life of individuals undergoing hemodialysis is related to many factors, including sleep disorders.</p><p><strong>Objective: </strong>To determine the prevalence of sleep disorders in individuals undergoing hemodialysis and to assess the effect of clinical and biochemical parameters on their sleep quality.</p><p><strong>Method: </strong>We conducted a cross-sectional descriptive survey in multiple hemodialysis centers. Individuals were interviewed using a questionnaire for obtaining general personal information, the Pittsburgh Sleep Quality Index (PSQI) for assessing sleep quality, and the Epworth Sleepiness Scale (ESS) for assessing excessive daytime sleepiness (EDS).</p><p><strong>Results: </strong>The study included 338 individuals with a median age of 55 years. Poor sleep quality (PSQ) and EDS were present in 41.4% and 6.5% of the individuals, respectively. The individuals' PSQI scores were significantly proportional to their ESS scores. Concerns about the disease and its treatment (60.7%) and poor physical conditions (35.7%) were major reported reasons for PSQ. In a logistic regression analysis, female gender, household income, ESS score, chronic heart failure, and creatinine and parathormone levels were found to be independent predictors of PSQ; household income, PSQ presence, parathormone and potassium levels, and urea reduction ratio were found to be independent predictors of EDS.</p><p><strong>Conclusion: </strong>Sleep disorders are common in individuals undergoing hemodialysis and are related to gender, comorbid diseases, poor economic state, altered biochemical values, dialysis timing, and concerns about the disease and its treatment. Improving these individuals' quality of life through adequate psychological and financial support and early diagnosis of sleep disorders should decrease their mortality and morbidity rates.</p>","PeriodicalId":50671,"journal":{"name":"Cognitive and Behavioral Neurology","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2021-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39394194","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}
引用次数: 2
The Impact of Primary Progressive Aphasia on Picture Naming and General Language Ability. 原发性进行性失语症对图片命名及一般语言能力的影响。
IF 1.4 4区 医学
Cognitive and Behavioral Neurology Pub Date : 2021-09-02 DOI: 10.1097/WNN.0000000000000275
Eleni Peristeri, Lambros Messinis, Mary H Kosmidis, Grigorios Nasios, Alexios-Fotios A Mentis, Vasileios Siokas, Athina-Maria Aloizou, Anastasios Kotrotsios, Maria Andreou, Efthimios Dardiotis
{"title":"The Impact of Primary Progressive Aphasia on Picture Naming and General Language Ability.","authors":"Eleni Peristeri,&nbsp;Lambros Messinis,&nbsp;Mary H Kosmidis,&nbsp;Grigorios Nasios,&nbsp;Alexios-Fotios A Mentis,&nbsp;Vasileios Siokas,&nbsp;Athina-Maria Aloizou,&nbsp;Anastasios Kotrotsios,&nbsp;Maria Andreou,&nbsp;Efthimios Dardiotis","doi":"10.1097/WNN.0000000000000275","DOIUrl":"https://doi.org/10.1097/WNN.0000000000000275","url":null,"abstract":"<p><strong>Background: </strong>Primary progressive aphasia (PPA) is a clinical syndrome that is characterized by progressive deterioration of language while other cognitive domains remain relatively intact. The extent to which print exposure and cortical volume atrophy jointly influence picture naming and general language ability in individuals with PPA remains underexplored.</p><p><strong>Objective: </strong>To investigate the language performance of individuals with the nonfluent variant of primary progressive aphasia (nfvPPA) and to explore the impact of print exposure and cortical volume atrophy on their language ability.</p><p><strong>Method: </strong>We compared 14 Greek individuals with nfvPPA and similar age, education, disease duration, and cognitive ability with age-, gender- and education-matched Greek controls on picture naming and on language tasks of the Boston Diagnostic Aphasia Examination-Short Form, including oral word reading, word and sentence repetition, complex ideational material, and reading comprehension. The effects of print exposure and left-hemisphere cortical volume on the individuals' language performance were estimated through stepwise regression models.</p><p><strong>Results: </strong>The language performance of the individuals with nfvPPA was affected by print exposure and cortical volume atrophy. Picture naming and word reading were affected by print exposure. The highest contributions of cortical volume atrophy were found for the repetition, complex ideational material, and reading comprehension tasks.</p><p><strong>Conclusion: </strong>Print exposure and cortical volume atrophy may help explain variability in the language performance of nfvPPA individuals with similar age, education, disease duration, and cognitive ability.</p>","PeriodicalId":50671,"journal":{"name":"Cognitive and Behavioral Neurology","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2021-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39394197","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}
引用次数: 1
Clinical Characterization of Atypical Primary Progressive Aphasia in a 3-Year Longitudinal Study: A Case Report. 非典型原发性进行性失语症3年纵向研究的临床特征:1例报告。
IF 1.4 4区 医学
Cognitive and Behavioral Neurology Pub Date : 2021-09-02 DOI: 10.1097/WNN.0000000000000273
Stefania Basilico, Andrea Ciricugno, Giorgio Gelosa, Francesca G Magnani, Lorena Mosca, Cristina Popescu, Valentina Garibotto, Maurizio Sberna, Eraldo Paulesu, Gabriella Bottini
{"title":"Clinical Characterization of Atypical Primary Progressive Aphasia in a 3-Year Longitudinal Study: A Case Report.","authors":"Stefania Basilico,&nbsp;Andrea Ciricugno,&nbsp;Giorgio Gelosa,&nbsp;Francesca G Magnani,&nbsp;Lorena Mosca,&nbsp;Cristina Popescu,&nbsp;Valentina Garibotto,&nbsp;Maurizio Sberna,&nbsp;Eraldo Paulesu,&nbsp;Gabriella Bottini","doi":"10.1097/WNN.0000000000000273","DOIUrl":"https://doi.org/10.1097/WNN.0000000000000273","url":null,"abstract":"<p><p>The logopenic variant of primary progressive aphasia (lvPPA) is the most recent variant of primary progressive aphasia (PPA) to be identified; thus far, it has been poorly investigated. Despite being typically associated with Alzheimer disease (AD), lvPPA has recently been linked to frontotemporal lobe degeneration (FTLD), with distinctive cognitive and neural features that are worthy of further investigation. Here, we describe the neuropsychological and linguistic profile, as well as cerebral abnormalities, of an individual exhibiting PPA and carrying a pathogenetic variant in the GRN gene, from a 3-year longitudinal perspective. The individual's initial profile resembled lvPPA because it was characterized by word-finding difficulties and phonological errors in spontaneous speech in addition to sentence repetition and phonological short-term memory impairments. The individual's structural and metabolic imaging data demonstrated left temporal and bilateral frontal atrophy and hypometabolism, respectively. On follow-up, as the pathology progressed, dysprosody, stereotypical speech patterns, agrammatism, and orofacial apraxia appeared, suggesting an overlap with the nonfluent variant of PPA (nfvPPA). Severe sentence comprehension impairment also became evident. Our longitudinal and multidisciplinary diagnostic approach allowed us to better characterize the progression of a GRN-positive lvPPA profile, providing neuropsychological and imaging indicators that might be helpful to improve classification between different PPA variants and to address a nosological issue. Finally, we discuss the importance of early diagnosis of PPA given the possible overlap between different PPA variants during the progression of the pathology.</p>","PeriodicalId":50671,"journal":{"name":"Cognitive and Behavioral Neurology","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2021-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39378553","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}
引用次数: 2
Preliminary Validation of the Learning Ratio for the HVLT-R and BVMT-R in Older Adults. 老年人HVLT-R和BVMT-R学习率的初步验证。
IF 1.4 4区 医学
Cognitive and Behavioral Neurology Pub Date : 2021-09-02 DOI: 10.1097/WNN.0000000000000277
Dustin B Hammers, Brian D Gradwohl, Amanda Kucera, Tracy J Abildskov, Elisabeth A Wilde, Robert J Spencer
{"title":"Preliminary Validation of the Learning Ratio for the HVLT-R and BVMT-R in Older Adults.","authors":"Dustin B Hammers,&nbsp;Brian D Gradwohl,&nbsp;Amanda Kucera,&nbsp;Tracy J Abildskov,&nbsp;Elisabeth A Wilde,&nbsp;Robert J Spencer","doi":"10.1097/WNN.0000000000000277","DOIUrl":"10.1097/WNN.0000000000000277","url":null,"abstract":"<p><strong>Background: </strong>The learning slope is typically represented as the raw difference between the final score and the score of the first learning trial. A new method for calculating the learning slope, the learning ratio (LR), was recently developed; it is typically represented as the number of items that are learned after the first trial divided by the number of items that are yet to be learned.</p><p><strong>Objective: </strong>To evaluate the convergent and criterion validity of the LR in order to understand its sensitivity to Alzheimer disease (AD) pathology.</p><p><strong>Method: </strong>Fifty-six patients from a memory clinic underwent standard neuropsychological assessment and quantitative brain imaging. LR scores were calculated from the Hopkins Verbal Learning Test-Revised and the Brief Visuospatial Memory Test-Revised and were compared with both standard memory measures and total hippocampal volumes, as well as between individuals with AD and those with mild cognitive impairment.</p><p><strong>Results: </strong>Lower LR scores were consistently associated with poorer performances on standard memory measures and smaller total hippocampal volumes, generally more so than traditional learning slope scores. The LR scores of the AD group were smaller than those of the group with mild cognitive impairment. Furthermore, the aggregation of LR scores into a single metric was partially supported.</p><p><strong>Conclusion: </strong>The LR is sensitive to AD pathology along the AD continuum. This result supports previous claims that the LR score can reflect learning capacity better than traditional learning calculations can by considering the amount of information that is learned at trial 1.</p>","PeriodicalId":50671,"journal":{"name":"Cognitive and Behavioral Neurology","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2021-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39394195","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}
引用次数: 2
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