When behavioral assessment detects frontotemporal dementia and cognitive testing does not: data from the Frontal Behavioral Inventory

2007 ◽  
Vol 22 (3) ◽  
pp. 266-267 ◽  
Author(s):  
Graziella Milan ◽  
Alessandro Iavarone ◽  
Elisa Lorè ◽  
Sara Vitaliano ◽  
Francesco Lamenza ◽  
...  
Keyword(s):  
Frontotemporal Dementia ◽  
Behavioral Assessment ◽  
Cognitive Testing

scholarly journals Young-onset frontotemporal dementia with FUS pathology

2020 ◽  
pp. practneurol-2020-002730
Author(s):  
Matthew Gowell ◽  
Ian Baker ◽  
Olaf Ansorge ◽  
Masud Husain
Keyword(s):  
Frontotemporal Dementia ◽  
Social Withdrawal ◽  
Behavioural Change ◽  
Executive Dysfunction ◽  
Cognitive Testing ◽  
Young Onset ◽  
Fused In Sarcoma ◽  
Severe Impairment ◽  
Characteristic Features ◽  
Relevant Family History

Frontotemporal dementia (FTD) is an uncommon cause of behavioural change in adults under the age of 50. A 44-year-old man presented with progressive neuropsychiatric disturbance characterised by social withdrawal, apathy, loss of empathy, motor stereotypies and hyperorality. Cognitive testing identified severe impairment, including executive dysfunction. MR scan of the brain showed bilateral symmetrical frontal atrophy. There was no relevant family history, and targeted genetic testing for FTD-associated variants in MAPT, GRN and C9orf72 genes proved negative. He became more withdrawn with disinhibited behaviour; his condition progressively worsened and he died 6 years later. The pathological diagnosis was frontotemporal lobar degeneration with fused-in-sarcoma (FUS) pathology, a rare sporadic cause of FTD, accounting for only 5%–10% of cases, its characteristic features including very young onset, motor stereotypies and hyperorality.

P3-352 The middelheim frontality score: a behavioral assessment scale that discriminates frontotemporal dementia from Alzheimer's disease

2004 ◽  
Vol 25 ◽  
pp. S455
Author(s):  
Peter Paul De Deyn ◽  
Sebastiaan Engelborghs ◽  
Jos Saerens ◽  
Johan Goeman ◽  
Peter Mariën ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Frontotemporal Dementia ◽  
Behavioral Assessment ◽  
Assessment Scale

scholarly journals A remote smartphone cognitive testing battery for frontotemporal dementia: Completion rate, reliability, and validity

2021 ◽  
Vol 17 (S6) ◽  
Author(s):  
Adam M. Staffaroni ◽  
Jack C Taylor ◽  
Annie L Clark ◽  
Hilary W. Heuer ◽  
Leah K. Forsberg ◽  
...  
Keyword(s):  
Frontotemporal Dementia ◽  
Reliability And Validity ◽  
Completion Rate ◽  
Cognitive Testing

scholarly journals Neuropathological and behavioral characterization of aged Grn R493X progranulin-deficient frontotemporal dementia knockin mice

2021 ◽  
Vol 9 (1) ◽  
Author(s):  
Jonathan Frew ◽  
Haakon Berge Nygaard
Keyword(s):  
Frontotemporal Dementia ◽  
Behavioral Assessment ◽  
Causal Variant ◽  
Nonsense Mutations ◽  
Clinical Presentations ◽  
Specific Increase ◽  
Male Specific ◽  
Behavioral Characterization ◽  
Relevant Outcome

AbstractFrontotemporal lobar degeneration (FTLD) causes a spectrum of clinical presentations of frontotemporal dementia (FTD), including progressive changes in behavior, personality, executive function, and language. Up to 20% of familial FTLD cases are caused by progranulin (GRN) haploinsufficiency (FTD-GRN), with one of the most common causal variant being a nonsense mutation at arginine 493 (R493X). Recently, a genetic knockin FTD-GRN mouse model was generated bearing this GrnR493X mutation, at the analogous arginine in murine Grn. Aged, homozygous GrnR493X mice (GrnR493X/R493X) have been shown to phenotypically replicate several neuropathological hallmarks previously demonstrated in Grn null mice. We conducted a comprehensive neuropathological and behavioral assessment of 18 month old GrnR493X/R493X mice, observing a striking lysosomal dysfunction and thalamic neurodegeneration not previously described in this model, as well as a male-specific increase in generalized anxiety. These findings provide additional phenotypic markers of pathogenesis in aged GrnR493X/R493X mice that will contribute to better defining mechanisms underlying FTD-GRN, and offer relevant outcome measures for preclinical efficacy testing of novel therapeutics that target nonsense mutations leading to this devastating disease.

Quantitative Measurements of Motor Function in Alzheimer’s Disease, Frontotemporal Dementia, and Dementia with Lewy Bodies: A Proof-of-Concept Study

2018 ◽  
Vol 46 (3-4) ◽  
pp. 168-179 ◽  
Author(s):  
Frederikke Jeppesen Kragh ◽  
Marie Bruun ◽  
Esben Budtz-Jørgensen ◽  
Lena Elisabeth Hjermind ◽  
Robin Schubert ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Frontotemporal Dementia ◽  
Dementia With Lewy Bodies ◽  
Lewy Bodies ◽  
Cross Sectional Study ◽  
Cognitive Testing ◽  
Motor Dysfunction ◽  
Cross Sectional ◽  
Quantitative Measurements

Background: This study examines the efficacy of using quantitative measurements of motor dysfunction, compared to clinical ratings, in Alzheimer’s disease (AD), frontotemporal dementia (FTD), and dementia with Lewy bodies (DLB). Methods: In this cross-sectional study, 49 patients with a diagnosis of AD (n = 17), FTD (n = 19), or DLB (n = 13) were included and underwent cognitive testing, clinical motor evaluation, and quantitative motor tests: pronation/supination hand tapping, grasping and lifting, and finger and foot tapping. Results: Our results revealed significantly higher Q-Motor values in pronation/supination and in grip lift force assessment in AD, FTD, and DLB compared to healthy controls (HC). Q-Motor values detected significant differences between AD and HC, while clinical ratings did not. Conclusion: Our results suggest that quantitative measurements provide more objective and sensitive measurements of motor dysfunction in dementia.

Behavioral Quantitation Is More Sensitive Than Cognitive Testing in Frontotemporal Dementia

2003 ◽  
Vol 17 (4) ◽  
pp. 223-229 ◽  
Author(s):  
Andrew Kertesz ◽  
Wilda Davidson ◽  
Patricia McCabe ◽  
David Munoz
Keyword(s):  
Frontotemporal Dementia ◽  
Cognitive Testing

The Impact of Subjective and Objective Hearing Loss on Cognition and Memory in Older Adults

2015 ◽  
Vol 20 (2) ◽  
pp. 49-57 ◽  
Author(s):  
Yvonne Rogalski ◽  
Amy Rominger
Keyword(s):  
Older Adults ◽  
Hearing Loss ◽  
Reading Aloud ◽  
Cognitive Testing ◽  
Professional Collaboration ◽  
Cognitive Screening ◽  
Speech Language Pathologist ◽  
Text Passage ◽  
The Impact ◽  
Audiologic Evaluation

For this exploratory cross-disciplinary study, a speech-language pathologist and an audiologist collaborated to investigate the effects of objective and subjective hearing loss on cognition and memory in 11 older adults without hearing loss (OAs), 6 older adults with unaided hearing loss (HLOAs), and 16 young adults (YAs). All participants received cognitive testing and a complete audiologic evaluation including a subjective questionnaire about perceived hearing difficulty. Memory testing involved listening to or reading aloud a text passage then verbally recalling the information. Key findings revealed that objective hearing loss and subjective hearing loss were correlated and both were associated with a cognitive screening test. Potential clinical implications are discussed and include a need for more cross-professional collaboration in assessing older adults with hearing loss.

Objective Assessment of Hearing in Children: Update on Procedures and Protocols

2011 ◽  
Vol 21 (2) ◽  
pp. 50-58
Author(s):  
James W. Hall ◽  
Anuradha R. Bantwal
Keyword(s):  
Psychosocial Development ◽  
Otoacoustic Emissions ◽  
Objective Assessment ◽  
Behavioral Assessment ◽  
Auditory Brainstem ◽  
Test Battery ◽  
Auditory Evoked Responses ◽  
Brainstem Response ◽  
Assessment Techniques ◽  
Pediatric Populations

Early identification and diagnosis of hearing loss in infants and young children is the first step toward appropriate and effective intervention and is critical for optimal communicative and psychosocial development. Limitations of behavioral assessment techniques in pediatric populations necessitate the use of an objective test battery to enable complete and accurate assessment of auditory function. Since the introduction of the cross-check principle 35 years ago, the pediatric diagnostic test battery has expanded to include, in addition to behavioral audiometry, acoustic immittance measures, otoacoustic emissions, and multiple auditory evoked responses (auditory brainstem response, auditory steady state response, and electrocochleography). We offer a concise description of a modern evidence-based audiological test battery that permits early and accurate diagnosis of auditory dysfunction.

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