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
Weitere Informationen

Publikationsverlauf

Publikationsdatum:
14. November 2013 (online)

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.

 
  • References

  • 1 Pick A. Uber die beziehungen der senilen hirnatrophie zur aphasie. Prager Medizinische Wochenschrift 1892; 17: 165-167
  • 2 Alzheimer A. Uber eigenartige krankheitsfalle des sparteren alters. Psychiatr Nervenkr Z Gesamte Neurol Psychiatr 1911; 4: 356-385
  • 3 Johnson JK, Diehl J, Mendez MF , et al. Frontotemporal lobar degeneration: demographic characteristics of 353 patients. Arch Neurol 2005; 62 (6) 925-930
  • 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
  • 5 Ratnavalli E, Brayne C, Dawson K, Hodges JR. The prevalence of frontotemporal dementia. Neurology 2002; 58 (11) 1615-1621
  • 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
  • 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
  • 8 Roberson ED, Hesse JH, Rose KD , et al. Frontotemporal dementia progresses to death faster than Alzheimer disease. Neurology 2005; 65 (5) 719-725
  • 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
  • 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
  • 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
  • 12 Kipps CM, Nestor PJ, Fryer TD, Hodges JR. Behavioural variant frontotemporal dementia: not all it seems?. Neurocase 2007; 13 (4) 237-247
  • 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
  • 14 Lomen-Hoerth C, Anderson T, Miller B. The overlap of amyotrophic lateral sclerosis and frontotemporal dementia. Neurology 2002; 59 (7) 1077-1079
  • 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
  • 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
  • 17 Thompson SA, Patterson K, Hodges JR. Left/right asymmetry of atrophy in semantic dementia: behavioral-cognitive implications. Neurology 2003; 61 (9) 1196-1203
  • 18 Seeley WW, Bauer AM, Miller BL , et al. The natural history of temporal variant frontotemporal dementia. Neurology 2005; 64 (8) 1384-1390
  • 19 Snowden JS, Thompson JC, Neary D. Knowledge of famous faces and names in semantic dementia. Brain 2004; 127 (Pt 4) 860-872
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 27 Clinical and neuropathological criteria for frontotemporal dementia. The Lund and Manchester Groups. J Neurol Neurosurg Psychiatry 1994; 57 (4) 416-418
  • 28 Neary D, Snowden JS, Gustafson L , et al. Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology 1998; 51 (6) 1546-1554
  • 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
  • 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
  • 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
  • 32 Brun A. Frontal lobe degeneration of non-Alzheimer type. I. Neuropathology. Arch Gerontol Geriatr 1987; 6 (3) 193-208
  • 33 Snowden J, Neary D, Mann D. Frontotemporal lobar degeneration: clinical and pathological relationships. Acta Neuropathol 2007; 114 (1) 31-38
  • 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
  • 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
  • 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
  • 37 Mackenzie IR, Neumann M, Baborie A , et al. A harmonized classification system for FTLD-TDP pathology. Acta Neuropathol 2011; 122 (1) 111-113
  • 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
  • 39 Munoz DG, Neumann M, Kusaka H , et al. FUS pathology in basophilic inclusion body disease. Acta Neuropathol 2009; 118 (5) 617-627
  • 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
  • 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
  • 42 Seelaar H, Kamphorst W, Rosso SM , et al. Distinct genetic forms of frontotemporal dementia. Neurology 2008; 71 (16) 1220-1226
  • 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
  • 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
  • 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
  • 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
  • 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
  • 48 Gydesen S, Brown JM, Brun A , et al. Chromosome 3 linked frontotemporal dementia (FTD-3). Neurology 2002; 59 (10) 1585-1594
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 60 Merrilees J. A model for management of behavioral symptoms in frontotemporal lobar degeneration. Alzheimer Dis Assoc Disord 2007; 21 (4) S64-S69