The frontotemporal dementia-motor neuron disease continuum (Q38778723)

6 September 2017

title

The frontotemporal dementia-motor neuron disease continuum (English)

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6 September 2017

main subject

frontotemporal dementia

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6 September 2017

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6 September 2017

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6 September 2017

James R Burrell

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Glenda M Halliday

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Matthew C Kiernan

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6 September 2017

John R Hodges

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6 September 2017

language of work or name

English

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publication date

14 March 2016

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6 September 2017

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6 September 2017

volume

388

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6 September 2017

issue

10047

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6 September 2017

page(s)

919-931

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Frontal lobe dementia and motor neuron disease

6 September 2017

cites work

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Familial amyotrophic lateral sclerosis with frontotemporal dementia is linked to a locus on chromosome 9p13.2-21.3.

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7 January 2021

Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS

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7 January 2021

A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD

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7 January 2021

Heritability in frontotemporal dementia: more missing pieces?

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7 January 2021

A pan-European study of the C9orf72 repeat associated with FTLD: geographic prevalence, genomic instability, and intermediate repeats

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7 January 2021

Cognitive decline and reduced survival in C9orf72 expansion frontotemporal degeneration and amyotrophic lateral sclerosis

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7 January 2021

Distinct clinical and pathological characteristics of frontotemporal dementia associated with C9ORF72 mutations

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7 January 2021

The widening spectrum of C9ORF72-related disease; genotype/phenotype correlations and potential modifiers of clinical phenotype

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7 January 2021

Corticobasal and ataxia syndromes widen the spectrum of C9ORF72 hexanucleotide expansion disease

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7 January 2021

Expanded C9ORF72 hexanucleotide repeat in depressive pseudodementia

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7 January 2021

Association between repeat sizes and clinical and pathological characteristics in carriers of C9ORF72 repeat expansions (Xpansize-72): a cross-sectional cohort study

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7 January 2021

Identical twins with the C9orf72 repeat expansion are discordant for ALS

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7 January 2021

Evidence for an oligogenic basis of amyotrophic lateral sclerosis

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7 January 2021

Motor neuron disease and frontotemporal dementia: sometimes related, sometimes not.

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7 January 2021

The epidemiology of ALS: a conspiracy of genes, environment and time

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7 January 2021

Motor neuron involvement in multisystem proteinopathy: implications for ALS

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7 January 2021

A familial ALS case carrying a novel p.G147C SOD1 heterozygous missense mutation with non-executive cognitive impairment

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7 January 2021

FUS mutations in amyotrophic lateral sclerosis: clinical, pathological, neurophysiological and genetic analysis

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7 January 2021

The heritability and genetics of frontotemporal lobar degeneration.

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7 January 2021

TARDBP mutations in motoneuron disease with frontotemporal lobar degeneration

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7 January 2021

Targeting RNA foci in iPSC-derived motor neurons from ALS patients with a C9ORF72 repeat expansion

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7 January 2021

A blinded international study on the reliability of genetic testing for GGGGCC-repeat expansions in C9orf72 reveals marked differences in results among 14 laboratories

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

The product of C9orf72, a gene strongly implicated in neurodegeneration, is structurally related to DENN Rab-GEFs

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

A C9orf72 promoter repeat expansion in a Flanders-Belgian cohort with disorders of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum: a gene identification study

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7 January 2021

RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense intervention

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7 January 2021

Modeling key pathological features of frontotemporal dementia with C9ORF72 repeat expansion in iPSC-derived human neurons

1 reference

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7 January 2021

Reduced C9orf72 protein levels in frontal cortex of amyotrophic lateral sclerosis and frontotemporal degeneration brain with the C9ORF72 hexanucleotide repeat expansion

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7 January 2021

Lack of C9ORF72 coding mutations supports a gain of function for repeat expansions in amyotrophic lateral sclerosis

1 reference

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7 January 2021

Discovery of Novel DENN Proteins: Implications for the Evolution of Eukaryotic Intracellular Membrane Structures and Human Disease

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7 January 2021

Genome-wide screen identifies rs646776 near sortilin as a regulator of progranulin levels in human plasma

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https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Deletion of C9ORF72 results in motor neuron degeneration and stress sensitivity in C. elegans

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Targeted degradation of sense and antisense C9orf72 RNA foci as therapy for ALS and frontotemporal degeneration

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

RAN proteins and RNA foci from antisense transcripts in C9ORF72 ALS and frontotemporal dementia

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Antisense transcripts of the expanded C9ORF72 hexanucleotide repeat form nuclear RNA foci and undergo repeat-associated non-ATG translation in c9FTD/ALS

1 reference

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7 January 2021

Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS

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7 January 2021

C9orf72 hypermethylation protects against repeat expansion-associated pathology in ALS/FTD.

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7 January 2021

Hypermethylation of the CpG island near the G4C2 repeat in ALS with a C9orf72 expansion

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7 January 2021

Reduced C9orf72 gene expression in c9FTD/ALS is caused by histone trimethylation, an epigenetic event detectable in blood

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7 January 2021

Hypermethylation of repeat expanded C9orf72 is a clinical and molecular disease modifier

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7 January 2021

Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

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7 January 2021

New criteria for frontotemporal dementia syndromes: clinical and pathological diagnostic implications

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7 January 2021

Classification of FTLD-TDP cases into pathological subtypes using antibodies against phosphorylated and non-phosphorylated TDP43.

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7 January 2021

TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia

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7 January 2021

Beyond the temporal pole: limbic memory circuit in the semantic variant of primary progressive aphasia

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7 January 2021

Significance and limitation of the pathological classification of TDP-43 proteinopathy

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7 January 2021

The neuropathology associated with repeat expansions in the C9ORF72 gene

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7 January 2021

Sequential distribution of pTDP-43 pathology in behavioral variant frontotemporal dementia (bvFTD).

1 reference

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7 January 2021

Stages of pTDP-43 pathology in amyotrophic lateral sclerosis

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7 January 2021

Amyotrophic lateral sclerosis: dash-like accumulation of phosphorylated TDP-43 in somatodendritic and axonal compartments of somatomotor neurons of the lower brainstem and spinal cord

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Ubiquitin-positive inclusions and progression of pathology in frontotemporal dementia and motor neurone disease identifies a group with mainly early pathology

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Amyotrophic lateral sclerosis--a model of corticofugal axonal spread

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Clinico-pathological features in amyotrophic lateral sclerosis with expansions in C9ORF72.

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https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

The syndrome of cognitive impairment in amyotrophic lateral sclerosis: a population-based study

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https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Cognitive correlates in amyotrophic lateral sclerosis: a population-based study in Italy

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https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Cognitive changes predict functional decline in ALS: a population-based longitudinal study

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https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Detecting frontotemporal dysfunction in ALS: utility of the ALS Cognitive Behavioral Screen (ALS-CBS).

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7 January 2021

The ALS-FTD-Q: a new screening tool for behavioral disturbances in ALS.

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https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

A novel tool to detect behavioural symptoms in ALS.

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https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Motor neuron dysfunction in frontotemporal dementia.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Clinically undetected motor neuron disease in pathologically proven frontotemporal lobar degeneration with motor neuron disease

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7 January 2021

Relation between cognitive dysfunction and pseudobulbar palsy in amyotrophic lateral sclerosis

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https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Amyotrophic lateral sclerosis patients show executive impairments on standard neuropsychological measures and an ecologically valid motor-free test of executive functions

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7 January 2021

Is language impairment more common than executive dysfunction in amyotrophic lateral sclerosis?

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Cognitive change in ALS: a prospective study

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https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Selective impairment of verb processing associated with pathological changes in Brodmann areas 44 and 45 in the motor neurone disease-dementia-aphasia syndrome

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Cognitive and behavioural deficits associated with the orbitomedial prefrontal cortex in amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Emotional perception deficits in amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Deficits in emotional and social cognition in amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Evidence of social understanding impairment in patients with amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Emotional responding in amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Emotional empathy in amyotrophic lateral sclerosis: a behavioural and voxel-based morphometry study.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Are amyotrophic lateral sclerosis patients cognitively normal?

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Neuropsychiatric changes precede classic motor symptoms in ALS and do not affect survival

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Caregiver burden in amyotrophic lateral sclerosis is more dependent on patients' behavioral changes than physical disability: a comparative study

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Neurobehavioral features in frontotemporal dementia with amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Frontotemporal dementia associated with the C9ORF72 mutation: a unique clinical profile

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Lessons of ALS imaging: Pitfalls and future directions - A critical review

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Grey and white matter changes across the amyotrophic lateral sclerosis-frontotemporal dementia continuum

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https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Multiple kernel learning captures a systems-level functional connectivity biomarker signature in amyotrophic lateral sclerosis

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https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Divergent brain network connectivity in amyotrophic lateral sclerosis

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7 January 2021

Brain network connectivity assessed using graph theory in frontotemporal dementia.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Multiparametric MRI study of ALS stratified for the C9orf72 genotype

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

The metabolic signature of C9ORF72-related ALS: FDG PET comparison with nonmutated patients

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Altered network connectivity in frontotemporal dementia with C9orf72 hexanucleotide repeat expansion

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Biomarkers in amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Screening for cognition and behaviour changes in ALS.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Changes in cognition and behaviour in amyotrophic lateral sclerosis: nature of impairment and implications for assessment

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https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

ALS-Plus syndrome: non-pyramidal features in a large ALS cohort.

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https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Long-term survival in amyotrophic lateral sclerosis: a population-based study

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Development and validation of pedigree classification criteria for frontotemporal lobar degeneration

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https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review): report of the Quality Standards Subcommittee of the Am

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https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Malnutrition at the time of diagnosis is associated with a shorter disease duration in ALS.

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

Body mass index delineates ALS from FTD: implications for metabolic health

1 reference

https://api.crossref.org/works/10.1016%2FS0140-6736%2816%2900737-6

7 January 2021

10.1016/S0140-6736(16)00737-6

Identifiers

DOI

1 reference

6 September 2017

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