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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
TERMINOLOGY
Abbreviations
- Frontotemporal lobar degeneration (FTLD)
- Clinical subtypes
- Behavioral variant frontotemporal dementia (bvFTD)
- Primary progressive aphasia syndromes (PPA)
- 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
- Pick disease no longer used
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
- Diehl-Schmid J et al: Imaging frontotemporal lobar degeneration. Curr Neurol Neurosci Rep. 14(10):489, 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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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
TERMINOLOGY
Abbreviations
- Frontotemporal lobar degeneration (FTLD)
- Clinical subtypes
- Behavioral variant frontotemporal dementia (bvFTD)
- Primary progressive aphasia syndromes (PPA)
- 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
- Pick disease no longer used
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
- Diehl-Schmid J et al: Imaging frontotemporal lobar degeneration. Curr Neurol Neurosci Rep. 14(10):489, 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
