Rapidly Progressive Alzheimer's Disease: A Multicenter Update

Schmidt, C. Max; Haı̈k, Stéphane; Satoh, Katsuya; Rábano, Alberto; Martinez‐Martín, Pablo; Roeber, Sigrun; Brandel, Jean‐Philippe; Calero, Miguel; Pedro‐Cuesta, Jesús de; Laplanche, Jean‐Louis; Hauw, J J; Kretzschmar, Hans; Zerr, Inga

doi:10.3233/jad-2012-120007

Cited by 58 publications

(62 citation statements)

References 18 publications

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“…Two distinct subtypes of dementia progression have been identified, especially in AD [47,54,58], cases having either a very short (median survival 10 months) or a significantly longer survival and which may reflect education level [18,21]. Short survival cases were also evident in the present sample of FTLD-TDP, nine cases surviving for two years or less.…”

Section: Discussionmentioning

confidence: 52%

Survival in the pre-senile dementia frontotemporal lobar degeneration with TDP-43 proteinopathy: effects of genetic, demographic and neuropathological variables

Armstrong

2016

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A b s t r a c t Factors associated with survival were studied in 84 neuropathologically documented cases of the pre-senile dementia frontotemporal dementia lobar degeneration (FTLD) with transactive response (TAR) DNA-binding protein of 43 kDa (TDP-43) proteinopathy (FTLD-TDP). Kaplan-Meier survival analysis estimated mean survival as 7.9 years (range: 1-19 years, SD = 4.64). Familial and sporadic cases exhibited similar survival, including progranulin (GRN) gene mutation cases. No significant differences in survival were associated with sex, disease onset, Braak disease stage, or disease subtype, but higher survival was associated with lower post-mortem brain weight. Survival was significantly reduced in cases with associated motor neuron disease (FTLD-MND) but increased with Alzheimer's disease (AD) or hippocampal sclerosis (HS) co-morbidity. Cox regression analysis suggested that reduced survival was associated with increased densities of neuronal cytoplasmic inclusions (NCI) while increased survival was associated with greater densities of enlarged neurons (EN) in the frontal and temporal lobes. The data suggest that: (1) survival in FTLD-

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Section: Discussionmentioning

confidence: 52%

Survival in the pre-senile dementia frontotemporal lobar degeneration with TDP-43 proteinopathy: effects of genetic, demographic and neuropathological variables

Armstrong

2016

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“…Recently it has been observed that patients suffering from rpAD present highly increased ptau levels in the CSF [146] when compared to controls and classical AD patients. Since it is estimated that rpAD may be accounting for 10-30% of all AD cases, it is urgently needed to establish if lack of disease stratification may lead to misinterpretation of p-tau analysis between rapidly progressive and classical AD forms.…”

Section: Phospho-taumentioning

confidence: 99%

New Frontiers in Alzheimer's Disease Diagnosis

Llorens¹,

Nuhn²,

Peter³

et al. 2015

Alzheimer's Disease - Challenges for the Future

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“…In a rapidly progressing form of AD that clinically may mimic CJD, the genetic profile (absence of ApoE ε4 homozygenity and biomarkers) differs from classical AD, suggesting that it might represent a distinct subtype of AD [209].…”

Section: Rapidly Progressive and Early Onset Dementiasmentioning

confidence: 99%

Neuropathology of Dementia Disorders

Jellinger¹

2014

J Alzheimers Dis Parkinsonism

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IntroductionDementia, encompassing deficits in several cognitive domains that are severe enough to interfere with daily functioning [1], in the new DSM V classification is classified within the broad category of major neurocognitive disorders, proposing specific criteria for the various etiologies [2]. Previously defined as the manifestation of deteriorating brain functions over time due to cell deaths in the brain caused by neurodegeneration or any other disease [3], according to recent research dementia is not primarily caused by neuronal cell death/loss, but by dysfunction and loss of synapses [4] in AD [5] and in α-synucleinopathies [6]. Other causes include cholinergic neuronal and axonal abnormalities [7,8], as well as pre-and postsynaptic cortical cholinergic deficits also occurring in early AD [9]. These changes due to disconnection of major nervous circuitries causing default networks [10][11][12][13] have been demonstrated in vivo in early AD [14], suggesting that disease progress is transmitted by neuronal pathways [15]. A prionlike spread of misfolded protein aggregates in the pathogenesis and progression of neurodegeneration is a hot spot of discussion [16][17][18][19][20][21].Both the prevalence and incidence of dementia increase exponentially with age. In 2010, 35.6 million people worldwide lived with dementia, with around 8 million new cases every year. Numbers are expected to double or triple every 5-10 years, to 135 millions in 2050, 16 millions in Europe. In 2010, 58% of all demented people lived in low-cost countries, with their proportion anticipated to rise to 71% in 2050 [22]. According to recent data from China the incidence of dementia was 9.87/1000 person years and the median standardised mortality ratio was 1.94:1 [23]. With the disproportional growth of the elderly population, dementia has become a major public health and socio-economic problem that threatens to become the scourge of our century. The total costs for dementia were US$ 604 billion in 2010 worldwide, up to 70% for social care alone [24], total payments for AD for 2013 were expected to be $ 203 billion in USA alone, not included contributions of unpaid caregivers [25]. Neuropathology of Dementia DisordersKurt A Jellinger* Institute of Clinical Neurobiology, Kenyongasse 18, A 1070, Vienna, Austria AbstractDementia, being not a specific disease but a syndrome characterized by deficits in several cognitive domains, is a major public health and socio-economic problem of our century. It is caused by dysfunction/loss of synapses and neurons inducing default neuronal networks. Despite updated concensus criteria for the clinical diagnosis of the major neurodegenerative disorders and new biomarkers, the diagnostic accuracy ranges from 65 to 96% (for Alzheimer's disease /AD), with a sensitivity versus other dementias of around 85.4% and a specificity of up to 77.7%. Pathologic assessment, using genetic and molecular biological methods, based on homogenous definitions, harmonized interlaboratory and assessment standards, can achiev...

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Rapidly Progressive Alzheimer's Disease: A Multicenter Update

Cited by 58 publications

References 18 publications

Survival in the pre-senile dementia frontotemporal lobar degeneration with TDP-43 proteinopathy: effects of genetic, demographic and neuropathological variables

Survival in the pre-senile dementia frontotemporal lobar degeneration with TDP-43 proteinopathy: effects of genetic, demographic and neuropathological variables

New Frontiers in Alzheimer's Disease Diagnosis

Neuropathology of Dementia Disorders

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