Frontotemporal Dementia
Frontotemporal Dementia is a topic covered in the Johns Hopkins Psychiatry Guide.
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DEFINITION
- Frontotemporal dementia (FTD) encompasses a group of neurodegenerative diseases characterized by progressive impairments in temperament, conduct, executive functions, and language.
- Three canonical syndromes are recognized:
- Behavior variant FTD (bvFTD)
- The most common variant, it is characterized by a progressive deterioration of conduct and cognition, in association with radiological evidence of frontal and/or anterior temporal atrophy or hypometabolism.[1]
- Two primary progressive aphasia (PPA)/language variants
- Primary non-fluent aphasia (PNFA)
- Presents with labored and agrammatic speech alongside impaired sentence comprehension, with sparing of word and object knowledge, in association with radiological evidence of left posterior fronto-insular atrophy or hypometabolism[2]
- Semantic dementia(SD)
- Manifests as fluent and vacuous speech, featuring dysnomia and agnosia for words and objects, in association with radiological evidence of focal anterior temporal atrophy or hypometabolism[2]
- Primary non-fluent aphasia (PNFA)
- Behavior variant FTD (bvFTD)
- Eventually all phenotypes converge on a global dementia state, in which severe disability is observed in every cognitive and functional domain.[3]
- Frontotemporal lobar degeneration (FTLD) refers to the histopathological state that underlies the clinical syndromes.
- Cognitive impairment due to frontotemporal dementia is classified under the neurocognitive disorders (NCDs) section of the Diagnostic and Statistical Manual of Mental Disorders, 5th Edition (DSM-5).[4]
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DEFINITION
- Frontotemporal dementia (FTD) encompasses a group of neurodegenerative diseases characterized by progressive impairments in temperament, conduct, executive functions, and language.
- Three canonical syndromes are recognized:
- Behavior variant FTD (bvFTD)
- The most common variant, it is characterized by a progressive deterioration of conduct and cognition, in association with radiological evidence of frontal and/or anterior temporal atrophy or hypometabolism.[1]
- Two primary progressive aphasia (PPA)/language variants
- Primary non-fluent aphasia (PNFA)
- Presents with labored and agrammatic speech alongside impaired sentence comprehension, with sparing of word and object knowledge, in association with radiological evidence of left posterior fronto-insular atrophy or hypometabolism[2]
- Semantic dementia(SD)
- Manifests as fluent and vacuous speech, featuring dysnomia and agnosia for words and objects, in association with radiological evidence of focal anterior temporal atrophy or hypometabolism[2]
- Primary non-fluent aphasia (PNFA)
- Behavior variant FTD (bvFTD)
- Eventually all phenotypes converge on a global dementia state, in which severe disability is observed in every cognitive and functional domain.[3]
- Frontotemporal lobar degeneration (FTLD) refers to the histopathological state that underlies the clinical syndromes.
- Cognitive impairment due to frontotemporal dementia is classified under the neurocognitive disorders (NCDs) section of the Diagnostic and Statistical Manual of Mental Disorders, 5th Edition (DSM-5).[4]
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Last updated: April 3, 2022
Citation
Morrow, Christopher, et al. "Frontotemporal Dementia." Johns Hopkins Psychiatry Guide, 2022. Johns Hopkins Guide, www.hopkinsguides.com/hopkins/view/Johns_Hopkins_Psychiatry_Guide/787108/3.1/Frontotemporal_Dementia.
Morrow C, McClam T, Onyike C. Frontotemporal Dementia. Johns Hopkins Psychiatry Guide. 2022. https://www.hopkinsguides.com/hopkins/view/Johns_Hopkins_Psychiatry_Guide/787108/3.1/Frontotemporal_Dementia. Accessed January 28, 2023.
Morrow, C., McClam, T., & Onyike, C. (2022). Frontotemporal Dementia. In Johns Hopkins Psychiatry Guide https://www.hopkinsguides.com/hopkins/view/Johns_Hopkins_Psychiatry_Guide/787108/3.1/Frontotemporal_Dementia
Morrow C, McClam T, Onyike C. Frontotemporal Dementia [Internet]. In: Johns Hopkins Psychiatry Guide. ; 2022. [cited 2023 January 28]. Available from: https://www.hopkinsguides.com/hopkins/view/Johns_Hopkins_Psychiatry_Guide/787108/3.1/Frontotemporal_Dementia.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC
T1 - Frontotemporal Dementia
ID - 787108
A1 - Morrow,Christopher,M.D.
AU - McClam,Tamela,M.D.
AU - Onyike,Chiadi,M.D.
Y1 - 2022/04/03/
BT - Johns Hopkins Psychiatry Guide
UR - https://www.hopkinsguides.com/hopkins/view/Johns_Hopkins_Psychiatry_Guide/787108/3.1/Frontotemporal_Dementia
DB - Johns Hopkins Guide
DP - Unbound Medicine
ER -