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Semin Neurol 2013; 33(04): 336-341
DOI: 10.1055/s-0033-1359316
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Frontotemporal Dementia

David C. Perry
1   Department of Neurology, University of California, San Francisco, California
,
Bruce L. Miller
1   Department of Neurology, University of California, San Francisco, California
› Institutsangaben
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Publikationsverlauf

Publikationsdatum:
14. November 2013 (online)

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Abstract

Frontotemporal dementia (FTD) encompasses several clinical syndromes that involve a progressive change in behavior and/or language; it is more common than Alzheimer's disease in early-onset dementia under the age of 60 years. In the behavioral variant of FTD (bvFTD) patients have social and emotional changes with prominent disinhibition, apathy, lack of empathy, changes in diet, and repetitive behaviors. Motor neuron disease or parkinsonism are seen in association with bvFTD. Frontal and/or temporal atrophy are often seen on structural brain imaging. Several pathological entities can cause bvFTD, and they are defined by the presence of specific abnormal protein accumulations. Most cases are characterized by accumulation of the proteins tau, TAR-DNA-binding protein-43 (TDP-43), and fused in sarcoma (FUS). Though most cases are sporadic, a variety of genes have been identified that cause autosomal dominant forms of FTD. The most common mutations occur in C9ORF72, MAPT, and GRN. No disease-modifying treatments have been currently identified, but limited evidence supports the use of antidepressants or neuroleptics in symptomatic management, and education regarding nonpharmacologic methods may be helpful to caregivers.

Keywords

frontotemporal dementia - motor neuron disease - Pick's disease - neurodegenerative disease
 

  • References

  • 1 Pick A. Uber die beziehungen der senilen hirnatrophie zur aphasie. Prager Medizinische Wochenschrift 1892; 17: 165-167
    PubMedGoogle Scholar
  • 2 Alzheimer A. Uber eigenartige krankheitsfalle des sparteren alters. Psychiatr Nervenkr Z Gesamte Neurol Psychiatr 1911; 4: 356-385
    PubMedGoogle Scholar
  • 3 Johnson JK, Diehl J, Mendez MF , et al. Frontotemporal lobar degeneration: demographic characteristics of 353 patients. Arch Neurol 2005; 62 (6) 925-930
    CrossrefPubMedGoogle Scholar
  • 4 Knopman DS, Petersen RC, Edland SD, Cha RH, Rocca WA. The incidence of frontotemporal lobar degeneration in Rochester, Minnesota, 1990 through 1994. Neurology 2004; 62 (3) 506-508
    CrossrefPubMedGoogle Scholar
  • 5 Ratnavalli E, Brayne C, Dawson K, Hodges JR. The prevalence of frontotemporal dementia. Neurology 2002; 58 (11) 1615-1621
    CrossrefPubMedGoogle Scholar
  • 6 Rosso SM, Donker Kaat L, Baks T , et al. Frontotemporal dementia in The Netherlands: patient characteristics and prevalence estimates from a population-based study. Brain 2003; 126 (Pt 9) 2016-2022
    CrossrefPubMedGoogle Scholar
  • 7 Mercy L, Hodges JR, Dawson K, Barker RA, Brayne C. Incidence of early-onset dementias in Cambridgeshire, United Kingdom. Neurology 2008; 71 (19) 1496-1499
    CrossrefPubMedGoogle Scholar
  • 8 Roberson ED, Hesse JH, Rose KD , et al. Frontotemporal dementia progresses to death faster than Alzheimer disease. Neurology 2005; 65 (5) 719-725
    CrossrefPubMedGoogle Scholar
  • 9 Gregory CA, Serra-Mestres J, Hodges JR. Early diagnosis of the frontal variant of frontotemporal dementia: how sensitive are standard neuroimaging and neuropsychologic tests?. Neuropsychiatry Neuropsychol Behav Neurol 1999; 12 (2) 128-135
    PubMedGoogle Scholar
  • 10 Hornberger M, Piguet O, Graham AJ, Nestor PJ, Hodges JR. How preserved is episodic memory in behavioral variant frontotemporal dementia?. Neurology 2010; 74 (6) 472-479
    CrossrefPubMedGoogle Scholar
  • 11 Pasquier F, Grymonprez L, Lebert F, Van der Linden M. Memory impairment differs in frontotemporal dementia and Alzheimer's disease. Neurocase 2001; 7 (2) 161-171
    CrossrefPubMedGoogle Scholar
  • 12 Kipps CM, Nestor PJ, Fryer TD, Hodges JR. Behavioural variant frontotemporal dementia: not all it seems?. Neurocase 2007; 13 (4) 237-247
    CrossrefPubMedGoogle Scholar
  • 13 Khan BK, Yokoyama JS, Takada LT , et al. Atypical, slowly progressive behavioural variant frontotemporal dementia associated with C9ORF72 hexanucleotide expansion. J Neurol Neurosurg Psychiatry 2012; 83 (4) 358-364
    CrossrefPubMedGoogle Scholar
  • 14 Lomen-Hoerth C, Anderson T, Miller B. The overlap of amyotrophic lateral sclerosis and frontotemporal dementia. Neurology 2002; 59 (7) 1077-1079
    CrossrefPubMedGoogle Scholar
  • 15 Murphy JM, Henry RG, Langmore S, Kramer JH, Miller BL, Lomen-Hoerth C. Continuum of frontal lobe impairment in amyotrophic lateral sclerosis. Arch Neurol 2007; 64 (4) 530-534
    CrossrefPubMedGoogle Scholar
  • 16 Lomen-Hoerth C, Murphy J, Langmore S, Kramer JH, Olney RK, Miller B. Are amyotrophic lateral sclerosis patients cognitively normal?. Neurology 2003; 60 (7) 1094-1097
    CrossrefPubMedGoogle Scholar
  • 17 Thompson SA, Patterson K, Hodges JR. Left/right asymmetry of atrophy in semantic dementia: behavioral-cognitive implications. Neurology 2003; 61 (9) 1196-1203
    CrossrefPubMedGoogle Scholar
  • 18 Seeley WW, Bauer AM, Miller BL , et al. The natural history of temporal variant frontotemporal dementia. Neurology 2005; 64 (8) 1384-1390
    CrossrefPubMedGoogle Scholar
  • 19 Snowden JS, Thompson JC, Neary D. Knowledge of famous faces and names in semantic dementia. Brain 2004; 127 (Pt 4) 860-872
    CrossrefPubMedGoogle Scholar
  • 20 Seeley WW, Crawford R, Rascovsky K , et al. Frontal paralimbic network atrophy in very mild behavioral variant frontotemporal dementia. Arch Neurol 2008; 65 (2) 249-255
    CrossrefPubMedGoogle Scholar
  • 21 Ishii K, Sakamoto S, Sasaki M , et al. Cerebral glucose metabolism in patients with frontotemporal dementia. J Nucl Med 1998; 39 (11) 1875-1878
    PubMedGoogle Scholar
  • 22 Hornberger M, Geng J, Hodges JR. Convergent grey and white matter evidence of orbitofrontal cortex changes related to disinhibition in behavioural variant frontotemporal dementia. Brain 2011; 134 (Pt 9) 2502-2512
    CrossrefPubMedGoogle Scholar
  • 23 Rosen HJ, Allison SC, Schauer GF, Gorno-Tempini ML, Weiner MW, Miller BL. Neuroanatomical correlates of behavioural disorders in dementia. Brain 2005; 128 (Pt 11) 2612-2625
    CrossrefPubMedGoogle Scholar
  • 24 Woolley JD, Gorno-Tempini ML, Seeley WW , et al. Binge eating is associated with right orbitofrontal-insular-striatal atrophy in frontotemporal dementia. Neurology 2007; 69 (14) 1424-1433
    CrossrefPubMedGoogle Scholar
  • 25 Whitwell JL, Sampson EL, Loy CT , et al. VBM signatures of abnormal eating behaviours in frontotemporal lobar degeneration. Neuroimage 2007; 35 (1) 207-213
    CrossrefPubMedGoogle Scholar
  • 26 Perry DC, Whitwell JL, Boeve BF , et al. Voxel-based morphometry in patients with obsessive-compulsive behaviors in behavioral variant frontotemporal dementia. Eur J Neurol 2012; 19 (6) 911-917
    CrossrefPubMedGoogle Scholar
  • 27 Clinical and neuropathological criteria for frontotemporal dementia. The Lund and Manchester Groups. J Neurol Neurosurg Psychiatry 1994; 57 (4) 416-418
    CrossrefPubMedGoogle Scholar
  • 28 Neary D, Snowden JS, Gustafson L , et al. Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology 1998; 51 (6) 1546-1554
    CrossrefPubMedGoogle Scholar
  • 29 Rascovsky K, Hodges JR, Kipps CM , et al. Diagnostic criteria for the behavioral variant of frontotemporal dementia (bvFTD): current limitations and future directions. Alzheimer Dis Assoc Disord 2007; 21 (4) S14-S18
    CrossrefPubMedGoogle Scholar
  • 30 Rascovsky K, Hodges JR, Knopman D , et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain 2011; 134 (Pt 9) 2456-2477
    CrossrefPubMedGoogle Scholar
  • 31 Lamarre AK, Rascovsky K, Bostrom A , et al. Interrater reliability of the new criteria for behavioral variant frontotemporal dementia. Neurology 2013; 80 (21) 1973-1977
    CrossrefPubMedGoogle Scholar
  • 32 Brun A. Frontal lobe degeneration of non-Alzheimer type. I. Neuropathology. Arch Gerontol Geriatr 1987; 6 (3) 193-208
    CrossrefPubMedGoogle Scholar
  • 33 Snowden J, Neary D, Mann D. Frontotemporal lobar degeneration: clinical and pathological relationships. Acta Neuropathol 2007; 114 (1) 31-38
    CrossrefPubMedGoogle Scholar
  • 34 Urwin H, Josephs KA, Rohrer JD , et al; FReJA Consortium. FUS pathology defines the majority of tau- and TDP-43-negative frontotemporal lobar degeneration. Acta Neuropathol 2010; 120 (1) 33-41
    CrossrefPubMedGoogle Scholar
  • 35 Knopman DS, Mastri AR, Frey II WH, Sung JH, Rustan T. Dementia lacking distinctive histologic features: a common non-Alzheimer degenerative dementia. Neurology 1990; 40 (2) 251-256
    CrossrefPubMedGoogle Scholar
  • 36 Cairns NJ, Bigio EH, Mackenzie IR , et al; Consortium for Frontotemporal Lobar Degeneration. Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar Degeneration. Acta Neuropathol 2007; 114 (1) 5-22
    CrossrefPubMedGoogle Scholar
  • 37 Mackenzie IR, Neumann M, Baborie A , et al. A harmonized classification system for FTLD-TDP pathology. Acta Neuropathol 2011; 122 (1) 111-113
    CrossrefPubMedGoogle Scholar
  • 38 Neumann M, Rademakers R, Roeber S, Baker M, Kretzschmar HA, Mackenzie IR. A new subtype of frontotemporal lobar degeneration with FUS pathology. Brain 2009; 132 (Pt 11) 2922-2931
    CrossrefPubMedGoogle Scholar
  • 39 Munoz DG, Neumann M, Kusaka H , et al. FUS pathology in basophilic inclusion body disease. Acta Neuropathol 2009; 118 (5) 617-627
    CrossrefPubMedGoogle Scholar
  • 40 Neumann M, Roeber S, Kretzschmar HA, Rademakers R, Baker M, Mackenzie IR. Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease. Acta Neuropathol 2009; 118 (5) 605-616
    CrossrefPubMedGoogle Scholar
  • 41 Goldman JS, Farmer JM, Wood EM , et al. Comparison of family histories in FTLD subtypes and related tauopathies. Neurology 2005; 65 (11) 1817-1819
    CrossrefPubMedGoogle Scholar
  • 42 Seelaar H, Kamphorst W, Rosso SM , et al. Distinct genetic forms of frontotemporal dementia. Neurology 2008; 71 (16) 1220-1226
    CrossrefPubMedGoogle Scholar
  • 43 Whitwell JL, Jack Jr CR, Boeve BF , et al. Voxel-based morphometry patterns of atrophy in FTLD with mutations in MAPT or PGRN. Neurology 2009; 72 (9) 813-820
    CrossrefPubMedGoogle Scholar
  • 44 Le Ber I, Camuzat A, Hannequin D , et al; French research network on FTD/FTD-MND. Phenotype variability in progranulin mutation carriers: a clinical, neuropsychological, imaging and genetic study. Brain 2008; 131 (Pt 3) 732-746
    CrossrefPubMedGoogle Scholar
  • 45 Snowden JS, Rollinson S, Thompson JC , et al. Distinct clinical and pathological characteristics of frontotemporal dementia associated with C9ORF72 mutations. Brain 2012; 135 (Pt 3) 693-708
    CrossrefPubMedGoogle Scholar
  • 46 Al-Sarraj S, King A, Troakes C , et al. p62 positive, TDP-43 negative, neuronal cytoplasmic and intranuclear inclusions in the cerebellum and hippocampus define the pathology of C9orf72-linked FTLD and MND/ALS. Acta Neuropathol 2011; 122 (6) 691-702
    CrossrefPubMedGoogle Scholar
  • 47 Ash PE, Bieniek KF, Gendron TF , et al. Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS. Neuron 2013; 77 (4) 639-646
    CrossrefPubMedGoogle Scholar
  • 48 Gydesen S, Brown JM, Brun A , et al. Chromosome 3 linked frontotemporal dementia (FTD-3). Neurology 2002; 59 (10) 1585-1594
    CrossrefPubMedGoogle Scholar
  • 49 Johnson JO, Mandrioli J, Benatar M , et al; ITALSGEN Consortium. Exome sequencing reveals VCP mutations as a cause of familial ALS. Neuron 2010; 68 (5) 857-864
    CrossrefPubMedGoogle Scholar
  • 50 Swartz JR, Miller BL, Lesser IM, Darby AL. Frontotemporal dementia: treatment response to serotonin selective reuptake inhibitors. J Clin Psychiatry 1997; 58 (5) 212-216
    CrossrefPubMedGoogle Scholar
  • 51 Ikeda M, Shigenobu K, Fukuhara R , et al. Efficacy of fluvoxamine as a treatment for behavioral symptoms in frontotemporal lobar degeneration patients. Dement Geriatr Cogn Disord 2004; 17 (3) 117-121
    CrossrefPubMedGoogle Scholar
  • 52 Moretti R, Torre P, Antonello RM, Cazzato G, Bava A. Frontotemporal dementia: paroxetine as a possible treatment of behavior symptoms. A randomized, controlled, open 14-month study. Eur Neurol 2003; 49 (1) 13-19
    CrossrefPubMedGoogle Scholar
  • 53 Lebert F, Stekke W, Hasenbroekx C, Pasquier F. Frontotemporal dementia: a randomised, controlled trial with trazodone. Dement Geriatr Cogn Disord 2004; 17 (4) 355-359
    CrossrefPubMedGoogle Scholar
  • 54 Moretti R, Torre P, Antonello RM, Cazzato G, Griggio S, Bava A. Olanzapine as a treatment of neuropsychiatric disorders of Alzheimer's disease and other dementias: a 24-month follow-up of 68 patients. Am J Alzheimers Dis Other Demen 2003; 18 (4) 205-214
    CrossrefPubMedGoogle Scholar
  • 55 Curtis RC, Resch DS. Case of Pick's central lobar atrophy with apparent stabilization of cognitive decline after treatment with risperidone. J Clin Psychopharmacol 2000; 20 (3) 384-385
    CrossrefPubMedGoogle Scholar
  • 56 Fellgiebel A, Müller MJ, Hiemke C, Bartenstein P, Schreckenberger M. Clinical improvement in a case of frontotemporal dementia under aripiprazole treatment corresponds to partial recovery of disturbed frontal glucose metabolism. World J Biol Psychiatry 2007; 8 (2) 123-126
    CrossrefPubMedGoogle Scholar
  • 57 Mendez MF, Shapira JS, McMurtray A, Licht E. Preliminary findings: behavioral worsening on donepezil in patients with frontotemporal dementia. Am J Geriatr Psychiatry 2007; 15 (1) 84-87
    CrossrefPubMedGoogle Scholar
  • 58 Vercelletto M, Boutoleau-Bretonnière C, Volteau C , et al; French research network on Frontotemporal dementia. Memantine in behavioral variant frontotemporal dementia: negative results. J Alzheimers Dis 2011; 23 (4) 749-759
    PubMedGoogle Scholar
  • 59 Boxer AL, Knopman DS, Kaufer DI , et al. Memantine in patients with frontotemporal lobar degeneration: a multicentre, randomised, double-blind, placebo-controlled trial. Lancet Neurol 2013; 12 (2) 149-156
    CrossrefPubMedGoogle Scholar
  • 60 Merrilees J. A model for management of behavioral symptoms in frontotemporal lobar degeneration. Alzheimer Dis Assoc Disord 2007; 21 (4) S64-S69
    CrossrefPubMedGoogle Scholar