Review: Clinical, genetic and neuroimaging features of frontotemporal dementia

  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},
  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… 
Systematic Review: Genetic, Neuroimaging, and Fluids Biomarkers for Frontotemporal Dementia Across Latin America Countries
There is an urgent need for harmonized, innovative, and cross-regional studies with a global perspective across multiple areas of dementia knowledge to contribute to a more comprehensive understanding of the disease and its associated biomarkers.
Diagnosis and treatment for frontotemporal degenerations
It seems appropriate to use memantin in patients with agrammatic variant of PPA, which is character-ized by a combination of cognitive, behavioral, and emotional-affective disorders.
Frontotemporal lobar degenerations: from basic science to clinical manifestations
FTLD is now used as an overarching pathological term for the description of a heterogeneous group of neurodegenerative diseases, which are linked together by a relatively selective involvement of the frontal and/ or temporal lobes, and the characteristic topographical distributions of the pathology determine the clinical repertoire.
Clinical Update on C9orf72: Frontotemporal Dementia, Amyotrophic Lateral Sclerosis, and Beyond.
This chapter will address all issues and summarize the recent updates about clinical aspects of C9orf72 disease, focusing on both the common and the less typical phenotypes.
A Clinicopathologic Study of Movement Disorders in Frontotemporal Lobar Degeneration
Despite the considerable overlap with atypical parkinsonism, a systematic characterization of the movement disorders associated with frontotemporal lobar degeneration (FTLD) is lacking.
Neurofilament Light Chain as Biomarker for Amyotrophic Lateral Sclerosis and Frontotemporal Dementia
Especially in ALS, NFL levels are generally stable over time, which, together with their correlation with progression rate, makes NFL an ideal pharmacodynamic biomarker for therapeutic trials and discusses unsolved issues and potential for future developments.
Underlying genetic variation in familial frontotemporal dementia: sequencing of 198 patients
Differences in Sex Distribution Between Genetic and Sporadic Frontotemporal Dementia
The higher male prevalence in sporadic bvFTD may provide important clues for its differential pathogenesis and warrants further research.
Uncovering the prevalence and neural substrates of anhedonia in frontotemporal dementia.
This is the first study to document the prevalence and neural correlates of anhedonia in FTD in comparison with Alzheimer's disease, and its potential overlap with related motivational symptoms including apathy and depression, and point to the importance of considering anhedonian as a primary presenting feature of behavioural variant FTD and semantic dementia.
Lateralization of Motor Signs Affects Symptom Progression in Parkinson Disease
Left side lateralization was associated with faster symptom progression and worse outcomes in multiple clinical domains in the authors' cohort, and clinicians should consider using motor predominance in their counseling regarding prognosis.


The clinical spectrum of sporadic and familial forms of frontotemporal dementia
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
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
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
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
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
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.
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.
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.
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.