Diagnostic Utility of Measuring Cerebral Atrophy in the Behavioral Variant of Frontotemporal Dementia and Association With Clinical Deterioration

Illán-Gala, Ignacio; Falgàs, Neus; Friedberg, Adit; Castro‐Suárez, Sheila; Keret, Ophir; Rogers, Nicole; Öz, Didem; Nigro, S. Ló; Quattrone, Andrea; Quattrone, Aldo; Wolf, Amy; Younes, Kyan; Santos‐Santos, Miguel A.; Borrego‐Écija, Sergi; Cobigo, Yann; Dols‐Icardo, Oriol; Lladó, Albert; Sánchez‐Valle, Raquel; Clarimón, Jordi; Blesa, Rafael; Alcolea, Daniel; Fortea, Juan; Lleó, Alberto; Grinberg, Lea T.; Spina, Salvatore; Kramer, Joel H.; Rabinovici, Gil D.; Boxer, Adam L.; Tempini, Maria Luisa Gorno; Miller, Bruce L.; Seeley, William W.; Rosen, Howard J.; Perry, David C.

doi:10.1001/jamanetworkopen.2021.1290

Cited by 17 publications

(15 citation statements)

References 54 publications

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“…This result is consistent with our previous observation in participants with bvFTD who developed PSP-RS during follow-up or had PSP or CBD on autopsy. 38 This result is also consistent with a previous study suggesting that PSP variants with prominent nonmotor signs at diagnosis may benefit from specific neuroimaging signatures, including cortical regions. 39 Taken together, our results support the view that the MRPI can be used to increase the diagnostic certainty of either PSP or CBD in participants presenting with PSP-RS or CBS.…”

Section: Discussionsupporting

confidence: 92%

Diagnostic Accuracy of Magnetic Resonance Imaging Measures of Brain Atrophy Across the Spectrum of Progressive Supranuclear Palsy and Corticobasal Degeneration

et al. 2022

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Key Points Question Can widely available atrophy measures on magnetic resonance imaging (MRI) increase diagnostic accuracy of progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD)? Findings In this diagnostic study of 326 participants, different methods for quantifying cerebral atrophy on MRI at first diagnosis were applied. The combination of cortical and subcortical measures of atrophy had excellent diagnostic accuracy for the differentiation between PSP, CBD, and other pathologies, even in the subgroup of participants who did not have a movement disorder at diagnosis. Meaning These findings suggest that structural MRI could be used to increase diagnostic certainty of underlying PSP and CBD in diverse clinically relevant scenarios.

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Section: Discussionsupporting

confidence: 92%

Diagnostic Accuracy of Magnetic Resonance Imaging Measures of Brain Atrophy Across the Spectrum of Progressive Supranuclear Palsy and Corticobasal Degeneration

et al. 2022

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“…However, we utilized the most sensitive brief cognitive and specialized neuropsychological tests that have been previously validated in our population at our clinic, MRI brain findings, and re-assessed the patients at 2-year follow-up to ensure that the diagnosis of bvFTD was accurate ( 44 , 78 , 79 ). These diagnostic criteria have been utilized in other studies of patients with bvFTD ( 80 , 81 ). Additionally, no bvFTD cases had temporo-parietal damage associated with frontal atrophy on MRI, a typical pattern of frontal variant AD ( 82 ), further supporting the correct classification of patients.…”

Section: Discussionmentioning

confidence: 99%

Social Cognition and Behavioral Assessments Improve the Diagnosis of Behavioral Variant of Frontotemporal Dementia in Older Peruvians With Low Educational Levels

et al. 2021

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Background: The behavioral variant of frontotemporal dementia (bvFTD), characterized by early behavioral abnormalities and late memory impairment, is a neurodegenerative disorder with a detrimental impact on patients and their caregivers. bvFTD is often difficult to distinguish from other neurodegenerative diseases, such as Alzheimer's disease (AD), using brief cognitive tests. Combining brief socio-cognitive and behavioral evaluations with standard cognitive testing could better discriminate bvFTD from AD patients. We sought to evaluate the diagnostic accuracy of brief socio-cognitive tests that may differentiate bvFTD and AD patients with low educational levels.Methods: A prospective study was performed on 51 individuals over the age of 50 with low educational levels, with bvFTD or AD diagnosed using published criteria, and who were receiving neurological care at a multidisciplinary neurology clinic in Lima, Peru, between July 2017 and December 2020. All patients had a comprehensive neurological evaluation, including a full neurocognitive battery and brief tests of cognition (Addenbrooke's Cognitive Examination version III, ACE-III), social cognition (Mini-social Cognition and Emotional Assessment, Mini-SEA), and behavioral assessments (Frontal Behavioral Inventory, FBI; Interpersonal Reactivity Index—Emphatic Concern, IRI-EC; IRI—Perspective Taking, IRI-PT; and Self-Monitoring Scale—revised version, r-SMS). Receiver operating characteristic (ROC) analysis to calculate the area under the curve (AUC) was performed to compare the brief screening tests individually and combined to the gold standard of bvFTD and AD diagnoses.Results: The AD group was significantly older than the bvFTD group (p < 0.001). An analysis of the discriminatory ability of the ACE-III to distinguish between patients with AD and bvFTD (AUC = 0.85) and the INECO Frontal Screening (IFS; AUC = 0.78) shows that the former has greater discriminatory ability. Social and behavioral cognition tasks were able to appropriately discriminate bvFTD from AD. The Mini-SEA had high sensitivity and high moderate specificity (83%) for discriminating bvFTD from AD, which increased when combined with the brief screening tests ACE-III and IFS. The FBI was ideal with high sensitivity (83%), as well as the IRI-EC and IRI-PT that also were adequate for distinguishing bvFTD from AD.Conclusions: Our study supports the integration of socio-behavioral measures to the standard global cognitive and social cognition measures utilized for screening for bvFTD in a population with low levels of education.

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“…There are several reports of patients with a clinical PSP phenotype associated with non-PSP pathologies such as cortico-basal degeneration pathology (CBD) [ 67 , 72 , 73 , 77 , 78 , 79 ], globular glial tauopathies [ 73 , 78 ], TDP-43 pathology [ 79 , 80 ], and alpha-synucleinopathies [ 77 , 78 , 81 , 82 ]. On the other hand, patients with post-mortem PSP diagnosis can at times present in the early stages with parkinsonian syndromes resembling PD or MSA [ 73 , 82 , 83 ], cortico-basal syndrome [ 67 , 72 ], behavioral variant of fronto-temporal dementia [ 67 , 72 , 84 , 85 , 86 ], primary progressive aphasia [ 67 , 87 ], and rarely amnestic syndrome [ 67 ]. In this context of clinico-pathological heterogeneity, a biomarker associated with the underlying pathology would be of extreme value to guide the clinical diagnosis and also the selection of patients for clinical trials with new therapies directed to molecular targets.…”

Section: Mr Planimetric Biomarkersmentioning

confidence: 99%

Magnetic Resonance Planimetry in the Differential Diagnosis between Parkinson’s Disease and Progressive Supranuclear Palsy

et al. 2022

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The clinical differential diagnosis between Parkinson’s disease (PD) and progressive supranuclear palsy (PSP) is often challenging. The description of milder PSP phenotypes strongly resembling PD, such as PSP-Parkinsonism, further increased the diagnostic challenge and the need for reliable neuroimaging biomarkers to enhance the diagnostic certainty. This review aims to summarize the contribution of a relatively simple and widely available imaging technique such as MR planimetry in the differential diagnosis between PD and PSP, focusing on the recent advancements in this field. The development of accurate MR planimetric biomarkers, together with the implementation of automated algorithms, led to robust and objective measures for the differential diagnosis of PSP and PD at the individual level. Evidence from longitudinal studies also suggests a role of MR planimetry in predicting the development of the PSP clinical signs, allowing to identify PSP patients before they meet diagnostic criteria when their clinical phenotype can be indistinguishable from PD. Finally, promising evidence exists on the possible association between MR planimetric measures and the underlying pathology, with important implications for trials with new disease-modifying target therapies.

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Diagnostic Utility of Measuring Cerebral Atrophy in the Behavioral Variant of Frontotemporal Dementia and Association With Clinical Deterioration

Cited by 17 publications

References 54 publications

Diagnostic Accuracy of Magnetic Resonance Imaging Measures of Brain Atrophy Across the Spectrum of Progressive Supranuclear Palsy and Corticobasal Degeneration

Diagnostic Accuracy of Magnetic Resonance Imaging Measures of Brain Atrophy Across the Spectrum of Progressive Supranuclear Palsy and Corticobasal Degeneration

Social Cognition and Behavioral Assessments Improve the Diagnosis of Behavioral Variant of Frontotemporal Dementia in Older Peruvians With Low Educational Levels

Magnetic Resonance Planimetry in the Differential Diagnosis between Parkinson’s Disease and Progressive Supranuclear Palsy

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