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Continuum (Minneap Minn)
2016[Apr]; 22
(2 Dementia
): 464-89
PMID27042904
show ga
PURPOSE OF REVIEW: This article reviews the common behavioral and cognitive
features of frontotemporal dementia (FTD) and related disorders as well as the
distinguishing clinical, genetic, and pathologic features of the most common
subtypes. RECENT FINDINGS: Advances in clinical phenotyping, genetics, and
biomarkers have enabled improved predictions of the specific underlying molecular
pathology associated with different presentations of FTD. Evaluation of large
international cohorts has led to recent refinements in diagnostic criteria for
several of the FTD subtypes. SUMMARY: The FTDs are a group of neurodegenerative
disorders featuring progressive deterioration of behavior or language and
associated pathology in the frontal or temporal lobes. Based on anatomic,
genetic, and neuropathologic categorizations, the six clinical subtypes of FTD or
related disorders are: (1) behavioral variant of FTD, (2) semantic variant
primary progressive aphasia, (3) nonfluent agrammatic variant primary progressive
aphasia, (4) corticobasal syndrome, (5) progressive supranuclear palsy, and (6)
FTD associated with motor neuron disease. Recognition and accurate diagnoses of
FTD subtypes will aid the neurologist in the management of patients with FTD.
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