Frontotemporal Dementia: A Clinical Review.

  title={Frontotemporal Dementia: A Clinical Review.},
  author={Harri Sivasathiaseelan and Charles R. Marshall and Jennifer L. Agustus and Elia Benhamou and Rebecca L. Bond and Janneke E P van Leeuwen and Chris JD. Hardy and Jonathan Daniel Rohrer and Jason D. Warren},
  journal={Seminars in neurology},
  volume={39 2},
Frontotemporal dementias are a clinically, neuroanatomically, and pathologically diverse group of diseases that collectively constitute an important cause of young-onset dementia. Clinically, frontotemporal dementias characteristically strike capacities that define us as individuals, presenting broadly as disorders of social behavior or language. Neurobiologically, these diseases can be regarded as "molecular nexopathies," a paradigm for selective targeting and destruction of brain networks by… 

Figures and Tables from this paper

Frontotemporal Dementia: Dilemma in Discrimination From Similarly Presenting Neurological and Psychiatric Conditions

The following case describes the clinical course of a 50-year-old male with a recent history of sporadic visual and auditory hallucinations, followed by a gradual decline in cognitive function including declining memory, apathy and behavioral disinhibition, and social functioning, which are suggestive of FTD-type.

The Link Between Frontotemporal Dementia and Autoimmunity: A Case Presentation and Literature Review

A patient with long-standing rheumatoid arthritis and antiphospholipid syndrome who developed frontotemporal dementia is examined, potentially as a result of the chronic inflammatory state.

A Spanish Neuropsychological Battery Discriminates Between the Behavioral Variant of Frontotemporal Dementia and Primary Progressive Aphasia in a Colombian Sample

This study supports that the combination of an individual test of executive function and language, MoCA's subtest, and performance errors as well have good accuracy to discriminate between bvFTD and PPA.

Primary Progressive Aphasia: Toward a Pathophysiological Synthesis

Critical issues around diagnosis of the three major PPA variants are reviewed, as well as considering ‘fragmentary’ syndromes, before discussing physiological phenotyping of proteinopathies across the PPA spectrum.

Suspecting dementia: canaries, chameleons and zebras

It is shown how clinical intuition reflects underlying signature profiles of brain involvement by the diseases that cause dementia and next steps that can be taken to define the diagnosis are suggested.

Fronto-temporal dementia: a case study and strategies and support for caregivers.

A case study illustrates care strategies and family education for patients with Behavioural variant fronto-temporal dementia when nursing home placement may not be required based on the individual's condition.

Comparative diagnosis interest of NfL and pNfH in CSF and plasma in a context of FTD–ALS spectrum

NfL, but not pNfH, appeared to be useful in a context of differential diagnosis between FTD and psychiatric patients, Nevertheless, pNFH seem more specific for the diagnosis and follow-up of motor impairments.



Mouse models of frontotemporal dementia: A comparison of phenotypes with clinical symptomatology

Neuroimaging in frontotemporal lobar degeneration—predicting molecular pathology

Frontotemporal lobar degeneration (FTLD) encompasses a group of diseases characterized by neuronal loss and gliosis of the frontal and temporal lobes. Almost all cases of FTLD can be classified into

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.

Phenotypic signatures of genetic frontotemporal dementia.

Genetic FTD is heterogeneous but certain phenotypic signatures of the major causative genes can be identified, and neuroimaging studies show that GRN and MAPT mutations have distinct patterns of atrophy--asymmetric fronto-temporo-parietal atrophy with GRN versus relatively symmetric medial temporal and orbitofrontal lobe atrophywith MAPT.

Clinical and neuropathological criteria for frontotemporal dementia. The Lund and Manchester Groups.

  • Medicine, Psychology
    Journal of neurology, neurosurgery, and psychiatry
  • 1994
The clinical and pathological criteria for frontotemporal dementia are described and future studies may indicate that there is a spectrum of non-specific histology involving both, or alternatively, that each may reflect a distinct process governed by different genetic or molecular mechanisms.

Molecular neuropathology of frontotemporal dementia: insights into disease mechanisms from postmortem studies

This review will describe the current understanding of the molecular basis of FTD, focusing on insights gained from the study of human postmortem tissue, as well as some of the current controversies.

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.

The clinical diagnosis of early-onset dementias: diagnostic accuracy and clinicopathological relationships.

Examination of subsyndromes of frontotemporal lobar degeneration showed a relatively predictable relationship between clinical diagnosis and pathological subtype, and showed that dementias can be distinguished in life with a high level of accuracy.

Advances in understanding the molecular basis of frontotemporal dementia

Recent advances in understanding the molecular aspects of FTD are highlighted, which will provide the basis for improved patient care through the development of more-targeted diagnostic tests and therapies.