Frontotemporal Dementia

Christopher Morrow, M.D., Tamela McClam, M.D., Chiadi Onyike‎, M.D.
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]
  • 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]
  • 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