Review: Clinical, genetic and neuroimaging features of frontotemporal dementia

@article{Convery2019ReviewCG,
  title={Review: Clinical, genetic and neuroimaging features of frontotemporal dementia},
  author={Rhian S. Convery and Simon Mead and Jonathan Daniel Rohrer},
  journal={Neuropathology and Applied Neurobiology},
  year={2019},
  volume={45},
  pages={18 - 6}
}
Frontotemporal dementia (FTD) is a heterogeneous group of disorders causing neurodegeneration within a network of areas centred on the frontal and temporal lobes. Clinically, patients present with behavioural symptoms (behavioural variant FTD) or language disturbance (primary progressive aphasia), although there is an overlap with motor neurone disease and atypical parkinsonian disorders. Whilst neuroimaging commonly reveals abnormalities in the frontal and temporal lobes, a closer review… 
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References

SHOWING 1-10 OF 120 REFERENCES
The clinical spectrum of sporadic and familial forms of frontotemporal dementia
TLDR
This review aims to clarify the often confusing terminology of FTD, and outline the various clinical features and diagnostic criteria of sporadic and familial FTD syndromes.
Advances in neuroimaging in frontotemporal dementia
TLDR
This review will cover the major imaging modalities currently used in research and clinical practice, focusing on the key insights they have provided into FTD, including the onset and evolution of pathological changes and also importantly their utility as biomarkers for disease detection and staging, differential diagnosis and measurement of disease progression.
Frontotemporal dementia (Pick’s disease): Clinical features and assessment
TLDR
The development of comprehensive caregiver-based neuropsychiatric instruments, neuropsychologic tasks sensitive to semantic memory and other key cognitive impairments, and functional and structural brain imaging represent significant advances in the field.
The heritability and genetics of frontotemporal lobar degeneration
TLDR
Heritability varied across the different clinical subtypes of FTLD with the behavioral variant being the most heritable and frontotemporal dementia–motor neuron disease and the language syndromes the least heritable.
Neuropsychiatric features of C9orf72-associated behavioral variant frontotemporal dementia and frontotemporal dementia with motor neuron disease
TLDR
The recent literature on C9orf72-associated FTD and ALS is reviewed with focus on the neuropsychiatric features associated with this mutation, as well as the experience at University of California, San Francisco, making comparison of results challenging.
New Perspective on Parkinsonism in Frontotemporal Lobar Degeneration
TLDR
There is a need to understand parkinsonism in FTLD in order to obtain a better understanding of the disease, and increased attention is needed on the subject.
Distinct clinical and pathological characteristics of frontotemporal dementia associated with C9ORF72 mutations.
TLDR
Mutations in the C9ORF72 gene may be a major cause not only of frontotemporal dementia with motor neuron disease but also of late onset psychosis, and the behavioural characteristics of patients with C9 ORF72 mutations are qualitatively distinct.
TDP-43 pathology in familial frontotemporal dementia and motor neuron disease without Progranulin mutations.
TLDR
There exists considerable clinical variation within families with FTD + MND, which may be determined by other genetic or environmental factors.
The overlapping syndromes of the pick complex.
TLDR
The experience with FTD in a clinical cohort, with high rate of autopsy confirmation is presented, and the majority of cases are tauopathies, the majority has been discovered to have a TDP-43 and most recently a FUS proteinopathy, shared with ALS, opening potential opportunities for pharmacological approaches to treatment.
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