Brain Spect Perfusion of Frontotemporal Dementia Associated With Motor Neuron Disease

Guedj, Eric; Ber, Isabelle Le; Lacomblez, L.; Dubois, Bruno; Verpillat, Patrice; Didic, Mira; Salachas, François; Véra, Pierre; Hannequin, Didier; Lotterie, Jean Albert; Puel, Michèle; Décousus, M.; Thomas‐Antérion, Catherine; Magne, C.; Vercelletto, Martine; Bernard, Anne‐Marie; Golfier, Véronique; Pasquier, J; Michel, B-F; Namer, Izzie Jacques; Sellal, François; Bochet, Jean; Volteau, Magali; Brice, Alexis; Meininger, Vincent; Habert, Marie Odile

doi:10.1212/01.wnl.0000266638.53185.e7

Cited by 20 publications

(9 citation statements)

References 7 publications

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“…In bvFTD patients, we observed hypoperfusion without reduced CT in posterior cingulate cortex bilaterally, right insula and right inferior temporal gyrus, all regions known from previous MRI studies to show progressive atrophy during the course of the disease (Du et al, 2007; Meyer et al, 2017; Tan et al, 2013). These findings are also in accordance with previous SPECT studies reporting hypoperfusion of the cingulate cortex and temporal regions in FTD (Guedj et al, 2007; McMurtray et al, 2006). Notably, we found that not only reduced CT, but also hypoperfusion in frontal and temporal regions without overlapping atrophy significantly correlated with greater cognitive impairment and behavioural symptoms in bvFTD, strengthening the hypothesis that hypoperfusion may be associated with clinically relevant regions of neurodegeneration.…”

Section: Discussionsupporting

confidence: 93%

Perfusion alterations converge with patterns of pathological spread in transactive response DNA-binding protein 43 proteinopathies

Ferraro

Jester²,

Olm

et al. 2018

Neurobiology of Aging

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Amyotrophic lateral sclerosis (ALS) and the behavioural variant of frontotemporal dementia (bvFTD) commonly share the presence of TDP-43 inclusions. Structural MRI studies demonstrated evidence for TDP-43 pathology spread, but while structural imaging usually reveals overt neuronal loss, perfusion imaging may detect more subtle neural activity alterations. We evaluated perfusion as an early marker for incipient pathology associated brain alterations in TDP-43 proteinopathies. Cortical thickness (CT) and perfusion measurements were obtained in ALS (N=18), pathologically and/or genetically confirmed bvFTD-TDP (N=12), and healthy controls (N=33). BvFTD showed reduced frontotemporal CT, hypoperfusion encompassing orbitofrontal and temporal cortices, and hyperperfusion in motor and occipital regions. ALS did not show reduced CT, but exhibited hypoperfusion in motor and temporal regions, and hyperperfusion in frontal and occipital cortices. Frontotemporal hypoperfusion and reduced CT correlated with cognitive and behavioural impairment as investigated using Mini Mental State Examination (MMSE) and Philadelphia Brief Assessment of Cognition (PBAC) in bvFTD, and hypoperfusion in motor regions correlated with motor disability as measured by the ALS Functional Rating Scale-Revised (ALSFRS-R) in ALS. Hypoperfusion marked early pathologically involved regions, while hyperperfusion characterized regions of late pathological involvement. Distinct perfusion patterns may provide early markers of pathology distribution in TDP-43 proteinopathies.

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Section: Discussionsupporting

confidence: 93%

Perfusion alterations converge with patterns of pathological spread in transactive response DNA-binding protein 43 proteinopathies

Ferraro

Jester²,

Olm

et al. 2018

Neurobiology of Aging

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“…[16][17][18] Typically, in FTD, there is an asymmetric or bilateral hypoperfusion of the frontal lobes as seen in our pathologic confirmed FTLD-MND patients, but regions, such as the temporal lobe, cingulate gyrus, insula cortex, and even brainstem, can be involved. 16,17,19,20 In DLB, the perfusion characteristics can be different from FTD as DLB patients tend to have occipital hypoperfusion, although other areas, such as the lateral parietal and temporal lobes, precuneus, 18,21,22 and frontal, 23 are reported to be involved. Difficulty in diagnosis will arise in any patient with DLB-like features who show frontal hypoperfusion suggesting FTD pathology.…”

Section: Discussionsupporting

confidence: 67%

Frontotemporal Dementia Mimicking Dementia With Lewy Bodies

Claassen

Parisi²,

Giannini³

et al. 2008

Cognitive and Behavioral Neurology

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“…These memory deficits correlated with precuneus hypoperfusion. 37,38 Sparing of the precentral gyrus in MAPT is in line with the absence of MND in FTLD-tau. 33,34 Atrophy and hypoperfusion of these cortical regions are frequently described in mild cognitive impairment and early-stage Alzheimer disease, 35,36 and in patients with GRN gene mutations.…”

Section: Discussionmentioning

confidence: 54%