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Frontotemporal Lobar Degeneration
Miral D. Jhaveri, MD; James M. Provenzale, MD
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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)
            • Frontotemporal dementia 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

            • Heterogenous 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

                                          1. Shivamurthy VK et al: Brain FDG PET and the diagnosis of dementia. AJR Am J Roentgenol. 204(1):W76-85, 2015
                                          2. Bott NT et al: Frontotemporal dementia: diagnosis, deficits and management. Neurodegener Dis Manag. 4(6):439-54, 2014
                                          3. Chare L et al: New criteria for frontotemporal dementia syndromes: clinical and pathological diagnostic implications. J Neurol Neurosurg Psychiatry. 85(8):865-70, 2014
                                          4. Diehl-Schmid J et al: Imaging frontotemporal lobar degeneration. Curr Neurol Neurosci Rep. 14(10):489, 2014
                                          5. Bhogal P et al: The common dementias: a pictorial review. Eur Radiol. 23(12):3405-17, 2013
                                          6. Risacher SL et al: Neuroimaging biomarkers of neurodegenerative diseases and dementia. Semin Neurol. 33(4):386-416, 2013
                                          7. 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
                                          8. Mackenzie IR et al: Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update. Acta Neuropathol. 119(1):1-4, 2010
                                          9. King RD et al: Characterization of atrophic changes in the cerebral cortex using fractal dimensional analysis. Brain Imaging Behav. 3(2):154-166, 2009
                                          10. Lindberg O et al: Cortical morphometric subclassification of frontotemporal lobar degeneration. AJNR Am J Neuroradiol. 30(6):1233-9, 2009
                                          11. 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
                                          12. Mihara M et al: Magnetic resonance spectroscopic study of Alzheimer's disease and frontotemporal dementia/Pick complex. Neuroreport. 17(4):413-6, 2006
                                          13. Whitwell JL et al: Magnetic resonance imaging signatures of tissue pathology in frontotemporal dementia. Arch Neurol. 62(9):1402-8, 2005
                                          Related Anatomy
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                                          Related Differential Diagnoses
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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)
                                                    • Frontotemporal dementia 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

                                                    • Heterogenous 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

                                                                                  1. Shivamurthy VK et al: Brain FDG PET and the diagnosis of dementia. AJR Am J Roentgenol. 204(1):W76-85, 2015
                                                                                  2. Bott NT et al: Frontotemporal dementia: diagnosis, deficits and management. Neurodegener Dis Manag. 4(6):439-54, 2014
                                                                                  3. Chare L et al: New criteria for frontotemporal dementia syndromes: clinical and pathological diagnostic implications. J Neurol Neurosurg Psychiatry. 85(8):865-70, 2014
                                                                                  4. Diehl-Schmid J et al: Imaging frontotemporal lobar degeneration. Curr Neurol Neurosci Rep. 14(10):489, 2014
                                                                                  5. Bhogal P et al: The common dementias: a pictorial review. Eur Radiol. 23(12):3405-17, 2013
                                                                                  6. Risacher SL et al: Neuroimaging biomarkers of neurodegenerative diseases and dementia. Semin Neurol. 33(4):386-416, 2013
                                                                                  7. 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
                                                                                  8. Mackenzie IR et al: Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update. Acta Neuropathol. 119(1):1-4, 2010
                                                                                  9. King RD et al: Characterization of atrophic changes in the cerebral cortex using fractal dimensional analysis. Brain Imaging Behav. 3(2):154-166, 2009
                                                                                  10. Lindberg O et al: Cortical morphometric subclassification of frontotemporal lobar degeneration. AJNR Am J Neuroradiol. 30(6):1233-9, 2009
                                                                                  11. 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
                                                                                  12. Mihara M et al: Magnetic resonance spectroscopic study of Alzheimer's disease and frontotemporal dementia/Pick complex. Neuroreport. 17(4):413-6, 2006
                                                                                  13. Whitwell JL et al: Magnetic resonance imaging signatures of tissue pathology in frontotemporal dementia. Arch Neurol. 62(9):1402-8, 2005