Frontotemporal dementia and related disorders: Deciphering the enigma

Research output: Contribution to journalArticle

135 Citations (Scopus)

Abstract

In the past century, particularly the last decade, there has been enormous progress in our understanding of frontotemporal dementia, a non-Alzheimer's type dementia. Large clinicopathological series have been published that have clearly demonstrated an overlap between the clinical syndromes subsumed under the term frontotemporal dementia and the progressive supranuclear palsy syndrome, corticobasal syndrome, and motor neuron disease. There have also been significant advancements in brain imaging, neuropathology, and molecular genetics that have led to different approaches to classification. Unfortunately, the field is complicated by a barrage of overlapping clinical syndromes and histopathological diagnoses that does not allow one to easily identify relations between individual clinical syndromic presentations and underlying neuropathology. This review deciphers this web of terminology and highlights consistent, and hence important, associations between individual clinical syndromes and neuropathology. These associations could ultimately allow the identification of appropriate patient phenotypes for future targeted treatments.

Original languageEnglish (US)
Pages (from-to)4-14
Number of pages11
JournalAnnals of Neurology
Volume64
Issue number1
DOIs
StatePublished - Jul 2008

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Frontotemporal Dementia
Progressive Supranuclear Palsy
Motor Neuron Disease
Terminology
Neuroimaging
Dementia
Molecular Biology
Phenotype
Neuropathology

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Frontotemporal dementia and related disorders : Deciphering the enigma. / Josephs, Keith Anthony.

In: Annals of Neurology, Vol. 64, No. 1, 07.2008, p. 4-14.

Research output: Contribution to journalArticle

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