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Frontotemporal Dementia
Akiva Mintz, MD, PhD, MHA, CFA; Bryan J. Neth, BS
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Definitions

            • Frontotemporal dementia (FTD): Progressive neurodegenerative disorder of frontal/anterior temporal lobes
              • Commonly characterized by behavioral/personality changes and impairments in other cognitive domains leading to functional decline
              • Previously referred to as Pick disease
                • Now only used to describe pathologically confirmed cases with Pick bodies

          IMAGING

          • Imaging Recommendations

            • Nuclear Medicine Findings

              • Artifacts and Quality Control

                DIFFERENTIAL DIAGNOSIS

                  PATHOLOGY

                  • General Features

                    CLINICAL ISSUES

                    • Presentation

                      • Demographics

                        • Natural History & Prognosis

                          • Treatment

                            Selected References

                            1. Motara H et al: Clinical impact and diagnostic accuracy of 2-[18F]-fluoro-2-deoxy-d-glucose positron-emission tomography/computed tomography (PET/CT) brain imaging in patients with cognitive impairment: a tertiary centre experience in the UK. Clin Radiol. 72(1):63-73, 2017
                            2. Vijverberg EG et al: Diagnostic accuracy of MRI and additional [18F]FDG-PET for behavioral variant frontotemporal dementia in patients with late onset behavioral changes. J Alzheimers Dis. 53(4):1287-97, 2016
                            3. Josephs KA et al: Neuropathological background of phenotypical variability in frontotemporal dementia. Acta Neuropathol. 122(2):137-53, 2011
                            4. Piguet O et al: Behavioural-variant frontotemporal dementia: diagnosis, clinical staging, and management. Lancet Neurol. 10(2):162-72, 2011
                            5. Seelaar H et al: Clinical, genetic and pathological heterogeneity of frontotemporal dementia: a review. J Neurol Neurosurg Psychiatry. 82(5):476-86, 2011
                            6. Zhang Y et al: White matter damage in frontotemporal dementia and Alzheimer's disease measured by diffusion MRI. Brain. 132(Pt 9):2579-92, 2009
                            7. Diehl-Schmid J et al: Decline of cerebral glucose metabolism in frontotemporal dementia: a longitudinal 18F-FDG-PET-study. Neurobiol Aging. 28(1):42-50, 2007
                            8. Foster NL et al: FDG-PET improves accuracy in distinguishing frontotemporal dementia and Alzheimer's disease. Brain. 130(Pt 10):2616-35, 2007
                            9. Mendez MF et al: Accuracy of the clinical evaluation for frontotemporal dementia. Arch Neurol. 64(6):830-5, 2007
                            10. Whitwell JL et al: Magnetic resonance imaging signatures of tissue pathology in frontotemporal dementia. Arch Neurol. 62(9):1402-8, 2005
                            11. Rosen HJ et al: Patterns of brain atrophy in frontotemporal dementia and semantic dementia. Neurology. 58(2):198-208, 2002
                            12. McKhann GM et al: Clinical and pathological diagnosis of frontotemporal dementia: report of the Work Group on Frontotemporal Dementia and Pick's Disease. Arch Neurol. 58(11):1803-9, 2001
                            13. Neary D et al: Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology. 51(6):1546-54, 1998
                            14. Ernst T et al: Frontotemporal dementia and early Alzheimer disease: differentiation with frontal lobe H-1 MR spectroscopy. Radiology. 203(3):829-36, 1997
                            Related Anatomy
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                            Related Differential Diagnoses
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                            References
                            Tables

                            Tables

                            KEY FACTS

                            • Terminology

                              • Imaging

                                • Pathology

                                  • Clinical Issues

                                    TERMINOLOGY

                                    • Definitions

                                      • Frontotemporal dementia (FTD): Progressive neurodegenerative disorder of frontal/anterior temporal lobes
                                        • Commonly characterized by behavioral/personality changes and impairments in other cognitive domains leading to functional decline
                                        • Previously referred to as Pick disease
                                          • Now only used to describe pathologically confirmed cases with Pick bodies

                                    IMAGING

                                    • Imaging Recommendations

                                      • Nuclear Medicine Findings

                                        • Artifacts and Quality Control

                                          DIFFERENTIAL DIAGNOSIS

                                            PATHOLOGY

                                            • General Features

                                              CLINICAL ISSUES

                                              • Presentation

                                                • Demographics

                                                  • Natural History & Prognosis

                                                    • Treatment

                                                      Selected References

                                                      1. Motara H et al: Clinical impact and diagnostic accuracy of 2-[18F]-fluoro-2-deoxy-d-glucose positron-emission tomography/computed tomography (PET/CT) brain imaging in patients with cognitive impairment: a tertiary centre experience in the UK. Clin Radiol. 72(1):63-73, 2017
                                                      2. Vijverberg EG et al: Diagnostic accuracy of MRI and additional [18F]FDG-PET for behavioral variant frontotemporal dementia in patients with late onset behavioral changes. J Alzheimers Dis. 53(4):1287-97, 2016
                                                      3. Josephs KA et al: Neuropathological background of phenotypical variability in frontotemporal dementia. Acta Neuropathol. 122(2):137-53, 2011
                                                      4. Piguet O et al: Behavioural-variant frontotemporal dementia: diagnosis, clinical staging, and management. Lancet Neurol. 10(2):162-72, 2011
                                                      5. Seelaar H et al: Clinical, genetic and pathological heterogeneity of frontotemporal dementia: a review. J Neurol Neurosurg Psychiatry. 82(5):476-86, 2011
                                                      6. Zhang Y et al: White matter damage in frontotemporal dementia and Alzheimer's disease measured by diffusion MRI. Brain. 132(Pt 9):2579-92, 2009
                                                      7. Diehl-Schmid J et al: Decline of cerebral glucose metabolism in frontotemporal dementia: a longitudinal 18F-FDG-PET-study. Neurobiol Aging. 28(1):42-50, 2007
                                                      8. Foster NL et al: FDG-PET improves accuracy in distinguishing frontotemporal dementia and Alzheimer's disease. Brain. 130(Pt 10):2616-35, 2007
                                                      9. Mendez MF et al: Accuracy of the clinical evaluation for frontotemporal dementia. Arch Neurol. 64(6):830-5, 2007
                                                      10. Whitwell JL et al: Magnetic resonance imaging signatures of tissue pathology in frontotemporal dementia. Arch Neurol. 62(9):1402-8, 2005
                                                      11. Rosen HJ et al: Patterns of brain atrophy in frontotemporal dementia and semantic dementia. Neurology. 58(2):198-208, 2002
                                                      12. McKhann GM et al: Clinical and pathological diagnosis of frontotemporal dementia: report of the Work Group on Frontotemporal Dementia and Pick's Disease. Arch Neurol. 58(11):1803-9, 2001
                                                      13. Neary D et al: Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology. 51(6):1546-54, 1998
                                                      14. Ernst T et al: Frontotemporal dementia and early Alzheimer disease: differentiation with frontal lobe H-1 MR spectroscopy. Radiology. 203(3):829-36, 1997