The prevalence of frontotemporal dementia

Ratnavalli, E.; Brayne, Carol; Dawson, Kate; Hodges, John R.

doi:10.1212/wnl.58.11.1615

Cited by 1,067 publications

(746 citation statements)

References 35 publications

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“…More than 6,000 diseases meet this definition, affecting 25 million Americans in total, and approximately 360 of these rare diseases are primarily neurological, including amyotrophic lateral sclerosis and Huntington disease (18,000-30,000 each), frontotemporal dementia (48,000), and myasthenia gravis (64,000), among many others. [42][43][44][45] The epidemiology and total costs of rare neurological diseases are poorly characterized, and no well-developed estimates are available. However, given the severity of many of these disorders and their aggregate burden, it is reasonable to assume substantial direct and indirect medical expenditures.…”

Section: Less Common Neurological Diseasesmentioning

confidence: 99%

The burden of neurological disease in theUnitedStates: A summary report and call to action

Gooch

Pracht

Borenstein

2017

Annals of Neurology

319

219

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Section: Less Common Neurological Diseasesmentioning

confidence: 99%

The burden of neurological disease in theUnitedStates: A summary report and call to action

Gooch

Pracht

Borenstein

2017

Annals of Neurology

319

219

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“…Frontotemporal lobar degeneration (FTLD) is the second most prevalent dementia in patients below 65 years old1, 2 and has the worst life expectancy among non‐prion dementia 3. Two main pathological subtypes have been described based on the proteinopathy found in the brain: around half of the cases develop aggregates of the microtubule‐associated protein tau (FTLD‐Tau), while the other half are characterized by cytoplasmic inclusions of the transactivator regulatory DNA‐binding protein 43 (TDP43, FTLD‐TDP) 4.…”

Section: Introductionmentioning

confidence: 99%

Novel CSF biomarkers to discriminate FTLD and its pathological subtypes

Campo

Galimberti

Elias

et al. 2018

Ann Clin Transl Neurol

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ObjectiveFrontotemporal lobar degeneration (FTLD) is the second most prevalent dementia in young patients and is characterized by the presence of two main protein aggregates in the brain, tau (FTLD‐Tau) or TDP43 (FTLD‐TDP), which likely require distinct pharmacological therapy. However, specific diagnosis of FTLD and its subtypes remains challenging due to largely overlapping clinical phenotypes. Here, we aimed to assess the clinical performance of novel cerebrospinal fluid (CSF) biomarkers for discrimination of FTLD and its pathological subtypes.Methods YKL40, FABP4, MFG‐E8, and the activities of catalase and specific lysosomal enzymes were analyzed in patients with FTLD‐TDP (n = 30), FTLD‐Tau (n = 20), AD (n = 30), DLB (n = 29), and nondemented controls (n = 29) obtained from two different centers. Models were validated in an independent CSF cohort (n = 188).Results YKL40 and catalase activity were increased in FTLD‐TDP cases compared to controls. YKL40 levels were also higher in FTLD‐TDP compared to FTLD‐Tau. We identified biomarker models able to discriminate FTLD from nondemented controls (MFG‐E8, tTau, and Aβ 42; 78% sensitivity and 83% specificity) and non‐FTLD dementia (YKL40, pTau, p/tTau ratio, and age; 90% sensitivity, 78% specificity), which were validated in an independent cohort. In addition, we identified a biomarker model differentiating FTLD‐TDP from FTLD‐Tau (YKL40, MFGE‐8, βHexA together with βHexA/tHex and p/tTau ratios and age) with 80% sensitivity and 82% specificity.InterpretationThis study identifies CSF protein signatures distinguishing FTLD and the two main pathological subtypes with optimal accuracy (specificity/sensitivity > 80%). Validation of these models may allow appropriate selection of cases for clinical trials targeting the accumulation of Tau or TDP43, thereby increasing their efficiency and facilitating the development of successful therapies.

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“…Frontotemporal lobar degeneration (FTLD) is the second most common form of neurodegenerative dementia in the presenile population and the third most prevalent form of neurodegenerative dementia overall 1, 2. With an estimated prevalence of 15/100,000 in the age group of 45–64 years, FTLD represents a significant challenge for social welfare 1, 3.…”

Section: Introductionmentioning

confidence: 99%

“…With an estimated prevalence of 15/100,000 in the age group of 45–64 years, FTLD represents a significant challenge for social welfare 1, 3. FTLD can present with a variety of syndromes, including progressive supranuclear palsy, corticobasal degeneration, and progressive aphasia, but the most common presentation is progressive change in personality with abnormalities in socioemotional behavior, referred to as the behavioral variant of frontotemporal dementia (bvFTD) 4.…”

Section: Introductionmentioning

confidence: 99%

Diagnostic utility of ASL‐MRI and FDG‐PET in the behavioral variant of FTD and AD

Tosun

Schuff

Rabinovici

et al. 2016

Ann Clin Transl Neurol

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ObjectiveTo compare the values of arterial spin‐labeled (ASL) MRI and fluorodeoxyglucose (FDG) PET in the diagnosis of behavioral variant of frontotemporal dementia (bvFTD) and Alzheimer's disease (AD).MethodsPartial least squares logistic regression was used to identify voxels with diagnostic value in cerebral blood flow (CBF) and cerebral metabolic rate of glucose (CMRgl) maps from patients with bvFTD (n = 32) and AD (n = 28), who were compared with each other and with cognitively normal controls (CN, n = 15). Diagnostic values of these maps were compared with each other.ResultsRegions that differentiated each disorder from controls were similar for CBF and CMRgl. For differentiating AD from CN, the areas under the curve (AUC) for CBF (0.89) and CMRgl (0.91) were similar, with similar sensitivity (CBF: 86%, CMRgl: 78%) and specificity (CBF: 92%, CMRgl: 100%). Likewise, for differentiating bvFTD from CN performances of CBF (AUC = 0.83) and CMRgl (AUC = 0.85) were equivalent, with similar sensitivity (CBF: 78%, CMRgl: 79%) and specificity (CBF: 92%, CMRgl: 100%). In differentiating bvFTD from AD, classification was again similar for CBF (AUC = 0.87) and CMRgl (AUC = 0.79), as were sensitivity (CBF: 83%, CMRgl: 89%) and specificity (CBF: 93%, CMRgl: 78%). None of the differences in any performance measure were statistically significant.InterpretationASL‐MRI has similar diagnostic utility as FDG‐PET in the diagnosis of AD and bvFTD. Continued development of ASL‐MRI as a diagnostic tool for neurodegenerative dementias is warranted.

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The prevalence of frontotemporal dementia

Abstract: Frontotemporal dementia is a more common cause of early-onset dementia than previously recognized and appears to be more common in men.

Cited by 1,067 publications

References 35 publications

The burden of neurological disease in theUnitedStates: A summary report and call to action

The burden of neurological disease in theUnitedStates: A summary report and call to action

Novel CSF biomarkers to discriminate FTLD and its pathological subtypes

Diagnostic utility of ASL‐MRI and FDG‐PET in the behavioral variant of FTD and AD

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