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Behavioural variant fronto-temporal dementia

Ananya Mahapatra, Mamta Sood, Roshan Bhad, Manjari Tripathi
Fronto-Temporal Dementia (FTD) is a cluster of syndromes, characterized by progressive deterioration of cognition, language and/or behavioural changes associated with degeneration of the frontal and temporal lobes. A 53-year-old man was admitted with a history of gradually progressive behavioural disturbances, disinhibition, unprovoked anger outbursts, apathy, disorganised behaviour and impaired self-care. A clinical diagnosis of Fronto temporal Dementia (behavioural variant) was made. Extensive investigations found no abnormality except in FDG-PET scan of the brain which revealed hypo metabolism in bilateral anterior insular region...
April 2016: Journal of Clinical and Diagnostic Research: JCDR
Chiara Cerami, Alessandra Dodich, Sandro Iannaccone, Alessandra Marcone, Giada Lettieri, Chiara Crespi, Luigi Gianolli, Stefano F Cappa, Daniela Perani
The behavioural variant of frontotemporal dementia (bvFTD) is a rare disease mainly affecting the social brain. FDG-PET fronto-temporal hypometabolism is a supportive feature for the diagnosis. It may also provide specific functional metabolic signatures for altered socio-emotional processing. In this study, we evaluated the emotion recognition and attribution deficits and FDG-PET cerebral metabolic patterns at the group and individual levels in a sample of sporadic bvFTD patients, exploring the cognitive-functional correlations...
2015: PloS One
Andrea Brioschi Guevara, Kristine M Knutson, Eric M Wassermann, Sarah Pulaski, Jordan Grafman, Frank Krueger
BACKGROUND: Theory of mind (ToM), the capacity to infer the intention, beliefs and emotional states of others, is frequently impaired in behavioural variant fronto-temporal dementia patients (bv-FTDp); however, its impact on caregiver burden is unexplored. SETTING: National Institute of Neurological Disorders and Stroke, National Institutes of Health. SUBJECTS: bv-FTDp (n = 28), a subgroup of their caregivers (n = 20) and healthy controls (n = 32)...
September 2015: Age and Ageing
Carlo Reverberi, Paolo Cherubini, Sara Baldinelli, Simona Luzzi
Semantic fluency is widely used both as a clinical test and as a basic tool for understanding how humans extract information from the semantic store. Recently, major efforts have been made to devise fine-grained scoring procedures to measure the multiple cognitive processes underlying fluency performance. Nevertheless, it is still unclear how many and which independent components are necessary to thoroughly describe performance on the fluency task. Furthermore, whether a combination of multiple indices can improve the diagnostic performance of the test should be assessed...
May 2014: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
Monica Ricci, Susanne Graef, Carlo Blundo, Laurie A Miller
In patients with focal lesions, patterns of learning, retrieval, and recognition deficits vary according to site of damage. Because different brain regions are affected by the underlying pathology in Alzheimer's dementia (AD) and behavioral variant fronto-temporal dementia (bvFTD), one might predict that the two disorders would result in different sorts of memory deficits on the Rey Auditory Verbal Learning Test (RAVLT). The aim of this investigation was to find a way to differentiate AD, bvFTD, and normal controls (NC) reliably based on RAVLT scores from retrospective samples of 82 Italian and 43 Australian participants...
2012: Clinical Neuropsychologist
Maria Pąchalska, Leszek Bidzan, Małgorzata Łukowicz, Mariola Bidzan, Katarzyna Markiewicz, Grażyna Jastrzębowska, Jan Talar
BACKGROUND: The aim of the paper is the differential diagnosis of various types of Fronto-Temporal Dementia (FTD), with the focus on its behavioural variant (bvFTD). MATERIAL/METHOD: Screening was done in order to assess the depth of dementia with the short version of MMSE, while evaluation of various variants of FTD was performed with the use of such neuropsychological tests as Newcomb and Chicago Fluency Tests, Wechsler Memory Scale-III (WMS-III), Western Aphasia Battery (WAB-R), and the Boston Naming Test (BNT)...
June 2011: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
David G Munoz, Manuela Neumann, Hirofumi Kusaka, Osamu Yokota, Kenji Ishihara, Seishi Terada, Shigetoshi Kuroda, Ian R Mackenzie
Basophilic Inclusion Body Disease (BIBD) is a tau-negative form of frontotemporal lobar degeneration (FTLD), characterized by neuronal cytoplasmic inclusions (NCI) that are visible on hematoxylin and eosin stain (HE), contain RNA, and are inconsistently ubiquitin-immunoreactive (ir). The normal nuclear expression of TDP-43 is not altered. Here we investigate whether the distribution of the structurally and functionally related protein fused in sarcoma (FUS) is altered in BIBD. Mutations in the FUS gene have recently been identified as a cause of familial amyotrophic lateral sclerosis (ALS)...
November 2009: Acta Neuropathologica
Teresa Torralva, Christopher M Kipps, John R Hodges, Luke Clark, Tristán Bekinschtein, María Roca, María Lujan Calcagno, Facundo Manes
Structural brain imaging and neuropsychological data implicate the orbital aspects of prefrontal cortex in the developing neuropathology of fvFTD. Damage to this region is associated with deficient performance on laboratory tasks assessing theory of mind (ToM) and affective decision-making (DM), but the relationship between these two capacities in patients with prefrontal cortex dysfunction is unclear. We studied a group of patients with early/mild fvFTD (n=20) and a group of matched normal controls (n=10) on the Iowa gambling task (IGT) of affective decision-making, and the "reading the mind in the eyes" (MIE) and "faux pas" (FP) tests of ToM...
January 28, 2007: Neuropsychologia
Christian Bocti, Conrad Rockel, Peter Roy, Fuqiang Gao, Sandra E Black
BACKGROUND/AIMS: Fronto-temporal dementia (FTD) designates a group of relatively common neurodegenerative disorders. The aim of this study was to characterize the patterns of brain atrophy in FTD compared to Alzheimer's disease (AD). METHODS: A novel semiautomatic volumetric MRI analysis method was applied to measure regional brain volumes in FTD (n = 15; behavioural variant n = 9, language variant n = 6) in contrast with AD patients (n = 15) and age-matched controls (NC) (n = 15)...
2006: Dementia and Geriatric Cognitive Disorders
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