Frontotemporal dementia

@article{Neary2005FrontotemporalD,
  title={Frontotemporal dementia},
  author={David Neary and Julie S. Snowden and David M A Mann},
  journal={The Lancet Neurology},
  year={2005},
  volume={4},
  pages={771-780}
}
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147 Citations
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12
Background Citations
61
Methods Citations
5
Results Citations
2

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147 Citations

Frontotemporal dementia parkinsonism: Clinical findings in a large Iranian family
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A male case with FTDP who presented with insidious onset of speech difficulty at a young age that was associated with signs of parkinsonism and a positive family history of FTD with MAPT gene mutation at exon 13 has been reported.
Frontotemporal dementia
Frontotemporal dementia is a clinicopathological syndrome caused by progressive degeneration of the frontal lobes, anterior temporal lobes or both. A wide spectrum of cognitive, psychological and…
Subcortical neurofibrillary tangles and argyrophilic grains in a case of familial frontotemporal dementia with parkinsonism.
[Overlapping features of frontotemporal dementia and amyotrophic lateral sclerosis].
TLDR
An update on the recent genetic and neuropathologic findings of ALS and FTD and a characterization of their clinical presentation forms, based on the current diagnostic criteria are provided, to provide an earlier diagnosis and a multidisciplinary care.
Frontotemporal Dementia and Psychiatric Symptoms
TLDR
A detailed history and psychiatric and neurologic examination with the usage of magnetic resonance imaging can help to distinguish FTD from other common forms of dementia and psychiatric disorders.
Newly Developed Interest at Drawing in a Patient with Frontotemporal Dementia.
TLDR
This is the first case report of a patient with a newfound interest in drawing after the onset of FTD in Korea.
Quejas de memoria en demencia frontotemporal variante conductual: a propósito de un caso
TLDR
The findings of the neuropsychological assessment confirm disturbances at the executive level with preservation of episodic memory, and imaging study show frontotemporal cortical atrophy of frontal predominance; results that suggest a probable behavioral variant of Frontotem temporal Dementia (FTD).
Pathogenic Huntingtin Repeat Expansions in Patients with Frontotemporal Dementia and Amyotrophic Lateral Sclerosis
TLDR
The findings reveal an etiological relationship between HTT repeat expansions and FTD/ALS syndromes and indicate that genetic screening of FTd/ALS patients for HTTrepeat expansions should be considered.
[Aphasia, prosopagnosia and mania: a case diagnosed with right temporal variant semantic dementia].
TLDR
A female patient was 46 years old, married, graduated from primary school, and had been admitted with complaints of hyperactivity, excessive talking, and decreased sleep for one week, report a frontotemporal dementia (FTD) case that referred with manic symptoms.
Frequency of progranulin mutations in a German cohort of 79 frontotemporal dementia patients
TLDR
This is the first published series of German FTD cases screened for progranulin mutations, and a sibling of one case presented with progressive nonfluent aphasia, clinically distinct from the brother.
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References

SHOWING 1-10 OF 145 REFERENCES
Autopsy proven sporadic frontotemporal dementia due to microvacuolar-type histology, with onset at 21 years of age
TLDR
This patient represents the youngest (so far) recorded case of FTD associated with this particular histological form of the disorder, and the underlying histology was of a microvacuolar-type degeneration.
Frontotemporal dementia with Pick-type histology associated with Q336R mutation in the tau gene.
TLDR
The mutation, Q336R, is indeed pathogenic and disease causing and not simply a coincidental rare and benign polymorphism and it has novel functional properties in vitro which are likely to be detrimental to neuronal function in vivo.
Clinical picture of frontal lobe degeneration of non-Alzheimer type.
TLDR
Frontal lobe degeneration of non-Alzheimer type (FLD) is the second most common primary degenerative dementia in southern Sweden and early recognition of FLD seems possible based on standardized clinical evaluation supported by neuropsychological tests, measurement of regional cerebral blood flow and other types of brain imaging.
A distinct familial presenile dementia with a novel missense mutation in the tau gene.
TLDR
This novel mutation causes a distinct familial tauopathy in a Japanese family with early onset hereditary frontotemporal dementia and a novel missense mutation (Ser305Asn) in the tau gene.
Familial progressive aphasia: its relationship to other forms of lobar atrophy.
TLDR
The differential anatomical atrophy supports the view that clinical manifestations of lobar atrophy are dictated by the topographical distribution of a common underlying pathology, linking the syndromes of progressive aphasia to dementia of frontal lobe type (DFT) and DFT with motor neuron disease.
Frontal lobe dementia and motor neuron disease.
  • P. Talbot
  • Psychology, Biology
    Journal of neural transmission. Supplementum
  • 1996
TLDR
The nosological status of FLD-MND remains enigmatic in the absence of defined pathological and molecular markers, whereas others suggest it represents an interface between FLD and "classic" (non-dementing) motor neuron disease (CMND).
Familial frontotemporal dementia and parkinsonism with a novel mutation at an intron 10+11‐splice site in the tau gene
TLDR
Although the patient had 2 novel point mutations of the tau gene, P301P (CCG to CCA) and an intron 10+11‐splice site (T to C), exon trapping analysis indicated that the latter was pathogenic.
Neuropathologic, biochemical, and molecular characterization of the frontotemporal dementias.
TLDR
It is found that the insoluble tau isoform patterns correlated strongly with the independently derived histopathologic diagnoses and forms a framework for further refinement of this classification scheme.
Frontotemporal lobar degeneration with motor neuron disease-type inclusions predominates in 76 cases of frontotemporal degeneration
TLDR
In this series of 76 cases, 29 (38%) were found to have frontotemporal lobar degeneration with motor neuron disease-type inclusions or FTLD-MND (with ALS), the most common neuropathological classification in this series.
Clinicopathological correlates in frontotemporal dementia
TLDR
The pathological substrate can be predicted in a significant proportion of FTD patients, which has important implications for studies targeting mechanistic treatments.
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