Semantic variant (sv-PPA): Previously known as semantic dementia
Nonfluent/agrammatic variant (nfv-PPA): Previously known as progressive nonfluent aphasia
Logopenic variant (lv-PPA): Has Alzheimer pathology & is not included as 1 of 3 clinical FTD syndromes
Frontotemporal dementia (FTD) with motor symptoms
Corticobasal degeneration (CBD)
Progressive supranuclear palsy (PSP)
FTD with motor neuron disease
FTD with amyotrophic lateral sclerosis (ALS)
Synonyms
Pick disease no longer used
Referred to pathologic variant with Pick bodies
Definitions
Heterogeneous family of neurodegenerative disorders characterized by focal lobar degeneration of frontal &/or temporal lobes
IMAGING
General Features
CT Findings
MR Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Reporting Tips
Selected References
Ishii K: Diagnostic imaging of dementia with Lewy bodies, frontotemporal lobar degeneration, and normal pressure hydrocephalus. Jpn J Radiol. 38(1):64-76, 2020
Raji CA et al: Overview of MR imaging volumetric quantification in neurocognitive disorders. Top Magn Reson Imaging. 28(6):311-5, 2019
Risacher SL et al: Neuroimaging in aging and neurologic diseases. Handb Clin Neurol. 167:191-227, 2019
Shepherd TM et al: Clinical use of integrated positron emission tomography-magnetic resonance imaging for dementia patients. Top Magn Reson Imaging. 28(6):299-310, 2019
Zukotynski K et al: PET/CT of dementia. AJR Am J Roentgenol. 211(2):246-59, 2018
Mann DM et al: Frontotemporal lobar degeneration: pathogenesis, pathology and pathways to phenotype. Brain Pathol. 27(6):723-36, 2017
Bang J et al: Frontotemporal dementia. Lancet. 386(10004):1672-82, 2015
Shivamurthy VK et al: Brain FDG PET and the diagnosis of dementia. AJR Am J Roentgenol. 204(1):W76-85, 2015
Bott NT et al: Frontotemporal dementia: diagnosis, deficits and management. Neurodegener Dis Manag. 4(6):439-54, 2014
Chare L et al: New criteria for frontotemporal dementia syndromes: clinical and pathological diagnostic implications. J Neurol Neurosurg Psychiatry. 85(8):865-70, 2014
Bhogal P et al: The common dementias: a pictorial review. Eur Radiol. 23(12):3405-17, 2013
Risacher SL et al: Neuroimaging biomarkers of neurodegenerative diseases and dementia. Semin Neurol. 33(4):386-416, 2013
Gorno-Tempini ML et al: Classification of primary progressive aphasia and its variants. Neurology. 76(11):1006-14, 2011
Rascovsky K et al: Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain. 134(Pt 9):2456-77, 2011
Krueger CE et al: Longitudinal rates of lobar atrophy in frontotemporal dementia, semantic dementia, and Alzheimer's disease. Alzheimer Dis Assoc Disord. 24(1):43-8, 2010
Mackenzie IR et al: Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update. Acta Neuropathol. 119(1):1-4, 2010
King RD et al: Characterization of atrophic changes in the cerebral cortex using fractal dimensional analysis. Brain Imaging Behav. 3(2):154-66, 2009
Lindberg O et al: Cortical morphometric subclassification of frontotemporal lobar degeneration. AJNR Am J Neuroradiol. 30(6):1233-9, 2009
Moon WJ et al: Atrophy measurement of the anterior commissure and substantia innominata with 3T high-resolution MR imaging: does the measurement differ for patients with frontotemporal lobar degeneration and Alzheimer disease and for healthy subjects? AJNR Am J Neuroradiol. 29(7):1308-13, 2008
Mihara M et al: Magnetic resonance spectroscopic study of Alzheimer's disease and frontotemporal dementia/Pick complex. Neuroreport. 17(4):413-6, 2006
Whitwell JL et al: Magnetic resonance imaging signatures of tissue pathology in frontotemporal dementia. Arch Neurol. 62(9):1402-8, 2005
Semantic variant (sv-PPA): Previously known as semantic dementia
Nonfluent/agrammatic variant (nfv-PPA): Previously known as progressive nonfluent aphasia
Logopenic variant (lv-PPA): Has Alzheimer pathology & is not included as 1 of 3 clinical FTD syndromes
Frontotemporal dementia (FTD) with motor symptoms
Corticobasal degeneration (CBD)
Progressive supranuclear palsy (PSP)
FTD with motor neuron disease
FTD with amyotrophic lateral sclerosis (ALS)
Synonyms
Pick disease no longer used
Referred to pathologic variant with Pick bodies
Definitions
Heterogeneous family of neurodegenerative disorders characterized by focal lobar degeneration of frontal &/or temporal lobes
IMAGING
General Features
CT Findings
MR Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Reporting Tips
Selected References
Ishii K: Diagnostic imaging of dementia with Lewy bodies, frontotemporal lobar degeneration, and normal pressure hydrocephalus. Jpn J Radiol. 38(1):64-76, 2020
Raji CA et al: Overview of MR imaging volumetric quantification in neurocognitive disorders. Top Magn Reson Imaging. 28(6):311-5, 2019
Risacher SL et al: Neuroimaging in aging and neurologic diseases. Handb Clin Neurol. 167:191-227, 2019
Shepherd TM et al: Clinical use of integrated positron emission tomography-magnetic resonance imaging for dementia patients. Top Magn Reson Imaging. 28(6):299-310, 2019
Zukotynski K et al: PET/CT of dementia. AJR Am J Roentgenol. 211(2):246-59, 2018
Mann DM et al: Frontotemporal lobar degeneration: pathogenesis, pathology and pathways to phenotype. Brain Pathol. 27(6):723-36, 2017
Bang J et al: Frontotemporal dementia. Lancet. 386(10004):1672-82, 2015
Shivamurthy VK et al: Brain FDG PET and the diagnosis of dementia. AJR Am J Roentgenol. 204(1):W76-85, 2015
Bott NT et al: Frontotemporal dementia: diagnosis, deficits and management. Neurodegener Dis Manag. 4(6):439-54, 2014
Chare L et al: New criteria for frontotemporal dementia syndromes: clinical and pathological diagnostic implications. J Neurol Neurosurg Psychiatry. 85(8):865-70, 2014
Bhogal P et al: The common dementias: a pictorial review. Eur Radiol. 23(12):3405-17, 2013
Risacher SL et al: Neuroimaging biomarkers of neurodegenerative diseases and dementia. Semin Neurol. 33(4):386-416, 2013
Gorno-Tempini ML et al: Classification of primary progressive aphasia and its variants. Neurology. 76(11):1006-14, 2011
Rascovsky K et al: Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain. 134(Pt 9):2456-77, 2011
Krueger CE et al: Longitudinal rates of lobar atrophy in frontotemporal dementia, semantic dementia, and Alzheimer's disease. Alzheimer Dis Assoc Disord. 24(1):43-8, 2010
Mackenzie IR et al: Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update. Acta Neuropathol. 119(1):1-4, 2010
King RD et al: Characterization of atrophic changes in the cerebral cortex using fractal dimensional analysis. Brain Imaging Behav. 3(2):154-66, 2009
Lindberg O et al: Cortical morphometric subclassification of frontotemporal lobar degeneration. AJNR Am J Neuroradiol. 30(6):1233-9, 2009
Moon WJ et al: Atrophy measurement of the anterior commissure and substantia innominata with 3T high-resolution MR imaging: does the measurement differ for patients with frontotemporal lobar degeneration and Alzheimer disease and for healthy subjects? AJNR Am J Neuroradiol. 29(7):1308-13, 2008
Mihara M et al: Magnetic resonance spectroscopic study of Alzheimer's disease and frontotemporal dementia/Pick complex. Neuroreport. 17(4):413-6, 2006
Whitwell JL et al: Magnetic resonance imaging signatures of tissue pathology in frontotemporal dementia. Arch Neurol. 62(9):1402-8, 2005
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