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.
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.
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.
Address correspondence to Dr Elizabeth Finger, LHSC Parkwood Institute, 550 Wellington Road, London, ON N6C 0A7, Canada, Elizabeth.firstname.lastname@example.org.
Relationship Disclosure: Dr Finger has received personal compensation as a speaker for Western University and receives grant support from the Canadian Institutes of Health Research for this work as well as grant funding from the Alzheimer Society of Canada, the Ministry of Research and Innovation, Ontario Brain Institute, and the Weston Foundation. Dr Finger has provided expert legal testimony for the Ontario Court of Justice.
Unlabeled Use of Products/Investigational Use Disclosure: Dr Finger discusses the unlabeled/investigational use of disease-modifying therapies in development, dopaminergic medications, neuroleptic medications, oxytocin, and selective serotonin reuptake inhibitors for the treatment of frontotemporal dementias.