Frontotemporal dementia

Česká verzia

Authors: R. Rusina ¹; R. Matěj ²; Z. Cséfalvay ³; J. Keller ⁴; V. Franková ⁵; M. Vyhnálek ^6,7
Authors place of work: Neurologická klinika 3. LF UK a Fakultní Thomayerova nemocnice, Praha ¹; Ústav patologie a molekulární medicíny, 3. LF UK a Fakultní Thomayerova nemocnice, Praha ²; Katedra logopedie, Komenského Univerzita, Bratislava, Slovensko ³; Neurologická klinika, 3. LF UK a FN Královské Vinohrady, Praha ⁴; Psychiatrická nemocnice v Dobřanech ⁵; Neurologická klinika 2. LF UK a FN Motol, Praha ⁶; Mezinárodní centrum klinického výzkumu, FN u sv. Anny, Brno ⁷
Published in the journal: Cesk Slov Neurol N 2021; 84/117(1): 9-29
Category: Minimonography
doi: https://doi.org/10.48095/cccsnn20219

Summary

This minimonography presents a comprehensive view of frontotemporal dementia. The behavioral variant of frontotemporal dementia (bvFTD) is a clinical syndrome within the spectrum of frontotemporal lobar degenerations. Typical manifestations of bvFTD include early behavioral disturbance, personality changes, cognitive deficits with a dominant impairment of executive functions, and language impairment. MRI typically shows asymmetric atrophy with frontal lobe predominance. The underlying neuropathology is in up to one half of the cases of a tauopathy, while the most frequent tau negative inclusions are TDP-43 protein deposits. Many patients with amyotrophic lateral sclerosis may develop cognitive and behavioral manifestation similar to bvFTD; these disorders have recently been called amyotrophic lateral sclerosis – frontotemporal spectrum disorder. In conclusion, the most important differential diagnose are discussed: frontal variant of Alzheimer’s disease and psychiatric disorders mimicking bvFTD.

Keywords:

Frontotemporal dementia – frontotemporal lobar degeneration – Alzheimer’s disease – behavioral variant of frontotemporal dementia – Pick’s disease – tauopathy – TDP-43 proteinopathy

Zdroje

1. Cséfalvay Z, Bajtošová R, Keller J. Primární progresivní afázie. Cesk Slov Neurol N 2020; 83/116 (3): 226–239. doi: 10.14735/amcsnn2020226.

2. Cairns NJ, Bigio EH, Mackenzie IR et al. Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar Degeneration. Acta Neuropathol 2007; 114 (1): 5–22. doi: 10.1007/s00401-007- 0237-2.

3. Rohan Z, Matej R. Current concepts in the classification and diagnosis of frontotemporal lobar degenerations: a practical approach. Arch Pathol Lab Med 2014; 138 (1): 132–138. doi: 10.5858/arpa.2012-0510-RS.

4. Mann DMA, Snowden JS. Frontotemporal lobar degeneration: Pathogenesis, pathology and pathways to phenotype. Brain Pathol 2017; 27 (6): 723–736. doi: 10.1111/bpa.12486.

5. Ferrari R, Manzoni C, Hardy J. Genetics and molecular mechanisms of frontotemporal lobar degeneration: an update and future avenues. Neurobiol Aging 2019; 78: 98–110. doi: 10.1016/j.neurobiolaging.2019.02. 006.

6. 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. doi: 10.1093/brain/awr179.

7. Vercelletto M, Boutoleau-Bretonnière C, Volteau C et al. Memantine in behavioral variant frontotemporal dementia: negative results. J Alzheimers Dis 2011; 23 (4): 749–759. doi: 10.3233/JAD-2010-101632.

8. Perry DC, Brown JA, Possin KL et al. Clinicopathological correlations in behavioural variant frontotemporal dementia. Brain 2017; 140 (12): 3329–3345. doi: 10.1093/brain/awx254.

9. Herrmann N, Black SE, Chow T et al. Serotonergic function and treatment of behavioral and psychological symptoms of frontotemporal dementia. Am J Geriatr Psychiatry 2012; 20 (9): 789–797. doi: 10.1097/JGP.0b013e31823033f3.

10. Kertesz A, McMonagle P, Blair M et al. The evolution and pathology of frontotemporal dementia. Brain 2005; 128 (Pt 9): 1996–2005. doi: 10.1093/brain/awh598.

11. Pick A. Über die Beziehungen der senilen Hirnatrophie zur Aphasie. Prager Med Wochenschr 1892; 17: 165–167.

12. Pick A, Berrios GE, Girling DM. On the relationship between senile cerebral atrophy and aphasia. Hist Psychiatry 1994; 5 (20): 542–547.

13. Pick A. Zur Symptomatologie der linksseitigen Schläfenlappenatrophie. Monatsschr Psychiatr Neurol 1904; 16: 378–388.

14. Pick A, Girling DM, Berrios GE. On the symptomatology of left- sided temporal lobe atrophy. Hist Psychiatry 1997; 8 (29): 149–59. doi: 10.1177/0957154X9700802910.

15. Ghetti B, Oblak AL, Boeve BF et al. Invited review: frontotemporal dementia caused by microtubule-associated protein tau gene (MAPT) mutations: a chameleon for neuropathology and neuroimaging. Neuropathol Appl Neurobiol 2015; 41 (1): 24–46. doi: 10.1111/nan.12213.

16. Rusina R, Matěj R, Růžička E et al. Diferenciální diag-nostika tauopatií – klinický pohled. Cesk Slov Neurol N 2015; 78/111 (5): 526–534. doi: 10.14735/amcsnn2015526.

17. Kovacs GG. Astroglia and tau: new perspectives. Front Aging Neurosci 2020; 12: 96. doi: 10.3389/fnagi. 2020.00096.

18. Neumann M, Sampathu DM, Kwong LK et al. Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science 2006; 314 (5796): 130–133. doi: 10.1126/science.1134108.

19. Neumann M, Mackenzie IR. Review: neuropathology of non-tau frontotemporal lobar degeneration. Neuropathol Appl Neurobiol 2019; 45 (1): 19–40. doi: 10.1111/nan.12526.

20. Kim G, Bolbolan K, Shahidehpour R et al. Morphology and distribution of TDP-43 pre-inclusions in primary progressive aphasia. J Neuropathol Exp Neurol 2019; 78 (3): 229–237. doi: 10.1093/jnen/nlz005.

21. Nelson PT, Dickson DW, Trojanowski JQ et al. Limbic-predominant age-related TDP-43 encephalopathy (LATE): consensus working group report. Brain 2019; 142 (6): 1503–1527. doi: 10.1093/brain/awz099.

22. Forman MS, Farmer J, Johnson JK et al. Frontotemporal dementia: clinicopathological correlations. Ann Neurol 2006; 59 (6): 952–962. doi: 10.1002/ana.20873.

23. Mercy L, Hodges JR, Dawson K et al. Incidence of early-onset dementias in Cambridgeshire, United Kingdom. Neurology 2008; 71 (19): 1496–1499. doi: 10.1212/01.wnl.0000334277.16896.fa.

24. Ratnavalli E, Brayne C, Dawson K et al. The prevalence of frontotemporal dementia. Neurology 2002; 58 (11): 1615–1621. doi: 10.1212/wnl.58.11.1615.

25. 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. doi: 10.1093/brain/awg204.

26. Hodges JR, Davies R, Xuereb J et al. Survival in frontotemporal dementia. Neurology 2003; 61 (3): 349–354. doi: 10.1212/01.wnl.0000078928.20107.52.

27. Vyhnalek M, Laczo J, Nikolai T et al. Neuropsychiatrie. In: Uhrova T, Roth J (eds). Neuropsychiatrie. Praha: Maxdorf 2020: 38–62.

28. Woolley JD, Khan BK, Murthy NK et al. The diagnostic challenge of psychiatric symptoms in neurodegenerative disease: rates of and risk factors for prior psychiatric diagnosis in patients with early neurodegenerative disease. J Clin Psychiatry 2011; 72 (2): 126–133. doi: 10.4088/JCP.10m06382oli.

29. Sommerlad A, Lee J, Warren J et al. Neurodegenerative disorder masquerading as psychosis in a forensic psychiatry setting. BMJ Case Rep 2014; bcr2013203458.doi: 10.1136/bcr-2013-203458.

30. Giovagnoli AR, Erbetta A, Reati F et al. Differential neuropsychological patterns of frontal variant frontotemporal dementia and Alzheimer‘s disease in a study of diagnostic concordance. Neuropsychologia 2008; 46 (5): 1495–1504. doi: 10.1016/j.neuropsychologia.2007.12. 023.

31. Mendez MF, Chen AK, Shapira JS et al. Acquired sociopathy and frontotemporal dementia. Dement Geriatr Cogn Disord 2005; 20 (2–3): 99–104. doi: 10.1159/000 086474.

32. Diehl-Schmid J, Perneczky R, Koch J et al. Guilty by suspicion? Criminal behavior in frontotemporal lobar degeneration. Cogn Behav Neurol 2013; 26 (2): 73–77. doi: 10.1097/WNN.0b013e31829cff11.

33. Le Ber I, Guedj E, Gabelle A et al. Demographic, neurological and behavioural characteristics and brain perfusion SPECT in frontal variant of frontotemporal dementia. Brain 2006; 129 (Pt 11): 3051–3065. doi: 10.1093/brain/awl288.

34. Zamboni G, Huey ED, Krueger F et al. Apathy and disinhibition in frontotemporal dementia: insights into their neural correlates. Neurology 2008; 71 (10): 736–742. doi: 10.1212/01.wnl.0000324920.96835.95.

35. Massimo L, Powers C, Moore P et al. Neuroanatomy of apathy and disinhibition in frontotemporal lobar degeneration. Dement Geriatr Cogn Disord 2009; 27 (1): 96–104. doi: 10.1159/000194658.

36. 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. doi: 10.1093/brain/awr173.

37. Kertesz A, Nadkarni N, Davidson W et al. The Frontal Behavioral Inventory in the differential diagnosis of frontotemporal dementia. J Int Neuropsychol Soc 2000; 6 (4): 460–468. doi: 10.1017/s1355617700644041.

38. de Vugt ME, Riedijk SR, Aalten P et al. Impact of behavioural problems on spousal caregivers: a comparison between Alzheimer’s disease and frontotemporal dementia. Dement Geriatr Cogn Disord 2006; 22 (1): 35–41. doi: 10.1159/000093102.

39. Merrilees J, Dowling GA, Hubbard E et al. Characterization of apathy in persons with frontotemporal dementia and the impact on family caregivers. Alzheimer Dis Assoc Disord 2013; 27 (1): 62–67. doi: 10.1097/WAD. 0b013e3182471c54.

40. McMurtray AM, Chen AK, Shapira JS et al. Variations in regional SPECT hypoperfusion and clinical features in frontotemporal dementia. Neurology 2006; 66 (4): 517–522. doi: 10.1212/01.wnl.0000197983.39436.e7.

41. Rankin KP, Gorno-Tempini ML, Allison SC et al. Structural anatomy of empathy in neurodegenerative disease. Brain 2006; 129 (Pt 11): 2945–2956. doi: 10.1093/brain/ awl254.

42. Eslinger PJ, Moore P, Anderson C et al. Social cognition, executive functioning, and neuroimaging correlates of empathic deficits in frontotemporal dementia. J Neuropsychiatry Clin Neurosci 2011; 23 (1): 74–82. doi: 10.1176/jnp.23.1.jnp74.

43. Mendez MF, Lauterbach EC, Sampson SM. An evidence-based review of the psychopathology of frontotemporal dementia: a report of the ANPA Committee on Research. J Neuropsychiatry Clin Neurosci 2008; 20 (2): 130–149. doi: 10.1176/jnp.2008.20.2.130.

44. Nyatsanza S, Shetty T, Gregory C et al. A study of stereotypic behaviours in Alzheimer’s disease and frontal and temporal variant frontotemporal dementia. J Neurol Neurosurg Psychiatry 2003; 74 (10): 1398–1402. doi: 10.1136/jnnp.74.10.1398.

45. Rosso SM, Roks G, Stevens M et al. Complex compulsive behaviour in the temporal variant of frontotemporal dementia. J Neurol 2001; 248 (11): 965–970. doi: 10.1007/s004150170049.

46. 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. doi: 10.1212/01.wnl.0000277461.06713.23.

47. Whitwell JL, Sampson EL, Loy CT et al. VBM signatures of abnormal eating behaviours in frontotemporal lobar degeneration. Neuroimage 2007; 35 (1): 207–213. doi: 10.1016/j.neuroimage.2006.12.006.

48. Piguet O, Petersen A, Yin Ka Lam B et al. Eating and hypothalamus changes in behavioral-variant frontotemporal dementia. Ann Neurol 2011; 69 (2): 312–319. doi: 10.1002/ana.22244.

49. Wittenberg D, Possin KL, Rascovsky K et al. The early neuropsychological and behavioral characteristics of frontotemporal dementia. Neuropsychol Rev 2008; 8 (1): 91–102. doi: 10.1007/s11065-008-9056-z.

50. Rascovsky K, Salmon DP, Hansen LA et al. Disparate letter and semantic category fluency deficits in autopsy-confirmed frontotemporal dementia and Alzheimer’s disease. Neuropsychology 2007; 21 (1): 20–30. doi: 10.1037/0894-4105.21.1.20.

51. Liscic RM, Storandt M, Cairns NJ et al. Clinical and psychometric distinction of frontotemporal and Alzheimer dementias. Arch Neurol 2007; 64 (4): 535–540. doi: 10.1001/archneur.64.4.535.

52. Ducharme S, Dols A, Laforce R et al. Recommendations to distinguish behavioural variant frontotemporal dementia from psychiatric disorders. Brain 2020; 143 (6): 1632–1650. doi: 10.1093/brain/awaa018.

53. 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. doi: 10.1001/archneurol.2007.38.

54. Neary D, Snowden JS, Gustafson L et al. Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology 1998; 51 (6): 1546–1554. doi: 10.1212/wnl.51.6.1546.

55. Lemos R, Duro D, Simões MR et al. The free and cued selective reminding test distinguishes frontotemporal dementia from Alzheimer‘s disease. Arch Clin Neuropsychol 2014; 29 (7): 670–679. doi: 10.1093/arclin/acu031.

56. Urbanová M, Vyhnálek M, Nikolai T et al. Validita testu Enhanced cued recall pro diferenciální diagnostiku demence Alzheimerova typu a behaviorální formy frontotemporální demence. Cesk Slov Neurol N 2014; 77/110 (5): 576–581.

57. Poos JM, Jiskoot LC, Papma JM et al. Meta-analytic review of memory impairment in behavioral variant frontotemporal dementia. J Int Neuropsychol Soc 2018; 24 (6): 593–605. doi: 10.1017/S1355617718000115.

58. Hornberger M, Piguet O. Episodic memory in frontotemporal dementia: a critical review. Brain 2012; 135 (Pt 3): 678–692. doi: 10.1093/brain/aws011.

59. Pennington C, Hodges JR, Hornberger M. Neural correlates of episodic memory in behavioral variant frontotemporal dementia. J Alzheimers Dis 2011; 24 (2): 261–268. doi: 10.3233/JAD-2011-101668.

60. Carlesimo GA, Perri R, Caltagirone C. Category cued recall following controlled encoding as a neuropsychological tool in the diagnosis of alzheimer’s disease: a review of the evidence. Neuropsychol Rev 2001; 21: 54–65. doi: 10.1007/s11065-010-9153-7.

61. Gregory C, Lough S, Stone V et al. Theory of mind in patients with frontal variant frontotemporal dementia and Alzheimer‘s disease: theoretical and practical implications. Brain 2002; 125 (Pt 4): 752–764. doi: 10.1093/brain/awf079.

62. Irish M, Hodges JR, Piguet O. Right anterior temporal lobe dysfunction underlies theory of mind impairments in semantic dementia. Brain 2014; 137 (Pt 4): 1241–1253. doi: 10.1093/brain/awu003.

63. Keane J, Calder AJ, Hodges JR et al. Face and emotion processing in frontal variant frontotemporal dementia. Neuropsychologia 2002; 40 (6): 655–665. doi: 10.1016/s0028-3932 (01) 00156-7.

64. Rosen HJ, Pace-Savitsky K, Perry RJ et al. Recognition of emotion in the frontal and temporal variants of frontotemporal dementia. Dement Geriatr Cogn Disord 2004; 17 (4): 277–281. doi: 10.1159/000077154.

65. O’Callaghan C, Bertoux M, Irish M et al. Fair play: social norm compliance failures in behavioural variant frontotemporal dementia. Brain 2016; 139 (Pt 1): 204–216. doi: 10.1093/brain/awv315.

66. Wong S, Irish M, O’Callaghan C et al. Should I trust you? Learning and memory of social interactions in dementia. Neuropsychologia 2017; 104: 157–167. doi: 10.1016/j.neuropsychologia.2017.08.016.

67. Ascher EA, Sturm VE, Seider BH et al. Relationship satisfaction and emotional language in frontotemporal dementia and Alzheimer disease patients and spousal caregivers. Alzheimer Dis Assoc Disord 2010; 24 (1): 49–55. doi: 10.1097/WAD.0b013e3181bd66a3.

68. Snowden J, Neary D, Mann D. Frontotemporal lobar degeneration: clinical and pathological relationships. Acta Neuropathol 2007; 114 (1): 31–38. doi: 10.1007/s00401-007-0236-3.

69. Kirschner HS. Frontotemporal dementia and primary progressive aphasia, a review. Neuropsychiatr Dis Treat 2014; 10: 1045–1055. doi: 10.2147/NDT.S38821.

70. Ogar JM, Dronkers NF, Brambati SM et al. Progressive nonfluent aphasia and its characteristic motor speech deficits. Alzheimer Dis Assoc Disord 2007; 21 (4): S23–S30. doi: 10.1097/WAD.0b013e31815d19fe.

71. Erkkinen MG, Zúñiga RG, Pardo CC et al. Artistic renaissance in frontotemporal dementia. JAMA 2018; 319 (13): 1304–1306. doi: 10.1001/jama.2017.19501.

72. Menšíková K, Tučková L, Kaňovský P. Atypický parkinsonizmus a frontotemporální demence – klinické, patologické a genetické aspekty. Cesk Slov Neurol N 2016; 79/112 (3): 275–286. doi: 10.14735/amcsnn2016275.

73. Burrell JR, Kiernan MC, Vucic S et al. Motor neuron dysfunction in frontotemporal dementia. Brain 2011; 134 (Pt 9): 2582–2594. doi: 10.1093/brain/awr195.

74. Tremolizzo L, Susani E, Aliprandi A et al. Muscle ultrasonography for detecting fasciculations in frontotemporal dementia. Amyotroph Lateral Scler Frontotemporal Degener 2014; 15 (7–8): 546–550. doi: 10.3109/ 21678421.2014.913636.

75. Perneczky R, Diehl-Schmid J, Förstl H et al. Urinary incontinence and its functional anatomy in frontotemporal lobar degenerations. Eur J Nucl Med Mol Imaging 2008; 35 (3): 605–610. doi: 10.1007/s00259-007-0626-8.

76. Kopecek M, Stepankova H, Lukavsky J et al. Montreal cognitive assessment (MoCA): Normative data for old and very old Czech adults. Appl Neuropsychol Adult 2017; 24 (1): 23–29. doi: 10.1080/23279095.2015.1065261.

77. Frontal Assessment Battery – Baterie frontálních funkcí. [online]. Dostupné z URL: https: //www.lf2.cuni.cz/files/page/files/2016/fab.doc.

78. Slachevsky A, Villalpando JM, Sarazin M et al. Frontal assessment battery and differential diagnosis of frontotemporal dementia and Alzheimer disease. Arch Neurol 2004; 61 (7): 1104–1107. doi: 10.1001/archneur.61.7.1104.

79. Dubois B, Slachevsky A, Litvan I et al. The FAB: a frontal assessment battery at bedside. Neurology 2000; 55 (11): 1621–2626. doi: 10.1212/wnl.55.11.1621.

80. Castiglioni S, Pelati O, Zuffi M et al. The frontal assessment battery does not differentiate frontotemporal dementia from Alzheimer‘s disease. Dement Geriatr Cogn Disord 2006; 22 (2): 125–131. doi: 10.1159/000093665.

81. Boban M, Malojcić B, Mimica N et al. The frontal assessment battery in the differential diagnosis of dementia. J Geriatr Psychiatry Neurol 2012; 25 (4): 201–207. doi: 10.1177/0891988712464821.

82. Harris JM, Gall C, Thompson JC et al. Sensitivity and specificity of FTDC criteria for behavioral variant frontotemporal dementia. Neurology 2013; 80 (20): 1881–1887. doi: 10.1212/WNL.0b013e318292a342.

83. Nikolai T, Štěpánková H, Michalec J et al. Testy verbální fluence, česká normativní studie pro osoby vyššího věku. Cesk Slov Neurol N 2015; 78/111 (3): 292–299. doi: 10.14735/amcsnn2015292.

84. Laisney M, Matuszewski V, Mézenge F et al. The underlying mechanisms of verbal fluency deficit in frontotemporal dementia and semantic dementia. J Neurol 2009; 256 (7): 1083–1094. doi: 10.1007/s00415-009- 5073-y.

85. Diehl-Schmid J, Pohl C, Ruprecht C et al. The Ekman 60 Faces Test as a diagnostic instrument in frontotemporal dementia. Arch Clin Neuropsychol 2007; 22 (4): 459–464. doi: 10.1016/j.acn.2007.01.024.

86. Torralva T, Roca M, Gleichgerrcht E et al. A neuropsychological battery to detect specific executive and social cognitive impairments in early frontotemporal dementia. Brain 2009; 132 (Pt 5): 1299–1309. doi: 10.1093/brain/awp041.

87. Barkhof F, Fox NC, Bastos-Leite AJ et al. Neuroimaging in dementia. Berlin: Springer Science & Business Media 2011.

88. Greaves CV, Rohrer JD. An update on genetic frontotemporal dementia. J Neurol 2019; 266 (8): 2075–2086. doi: 10.1007/s00415-019-09363-4.

89. Sellami L, Saracino D, Le Ber I. Genetic forms of frontotemporal lobar degeneration: current diagnostic approach and new directions in therapeutic strategies. Rev Neurol (Paris) 2020; 176 (7–8): 571–581. doi: 10.1016/j.neurol.2020.02.008.

90. Frontotemporal dementia. [online]. Available from URL: https: //omim.org/entry/600274?search=FTLD&highlight=ftld.

91. Van Mossevelde S, Engelborghs S, van der Zee J et aj. Genotype-phenotype links in frontotemporal lobar degeneration. Nat Rev Neurol 2018; 14 (6): 363–378. doi: 10.1038/s41582-018-0009-8.

92. Parobková E, Rusina R, Matějčková M et al. Genetika neurodegenerativních demencí v deseti bodech – co může neurolog očekávat od molekulárního genetika? Cesk Slov Neurol N 2019; 82 (1): 100–105 doi: 10.14735/amcsnn2019100.

93. Rohan Z, Rusina R, Matěj Z. Neuropatologické post mortem vyšetření mozku a míchy v deseti bodech – co může neurolog od neuropatologa očekávat při verifikaci klinické diagnózy neurodegenerativního onemocnění? Cesk Slov Neurol N 2018; 81 (1): 109–114. doi: 10.14735/amcsnn2018109.

94. Mendez MF, Shapira JS, McMurtray A et al. Preliminary findings: behavioral worsening on donepezil in patients with frontotemporal dementia. Am J Geriatr Psychiatry 2007; 5 (1): 84–87. doi: 10.1097/01.JGP.0000231744.69631. 33.

95. Boxer AL, Knopman DS, Kaufer DI et al. Memantine in patients with frontotemporal lobar degeneration: a multicentre, randomised, doubleblind, placebo-controlled trial. Lancet Neurol 2013; 12 (2): 149–156. doi: 10.1016/S1474-4422 (12) 70320-4.

96. Lebert F, Stekke W, Hasenbroekx C et al. Frontotemporal dementia: a randomised, controlled trial with trazodone. Dement Geriatr Cogn Disord 2004; 17 (4): 355–359. doi: 10.1159/000077171.

97. van Dyck CH, Arnsten AFT, Padala PR et al. Neurobiologic rationale for treatment of apathy in Alzheimer‘s disease with methylphenidate. Am J Geriatr Psychiatry 2020; 29 (1): 51–62. doi: 10.1016/j.jagp.2020.04.026.

98. Strong MJ, Abrahams S, Goldstein LH et al. Amyotrophic lateral sclerosis – frontotemporal spectrum disorder (ALS-FTSD): revised diagnostic criteria. Amyotroph Lateral Scler Frontotempral Degener 2017; 18 (3–4): 153–174. doi: 10.1080/21678421.2016.1267768.

99. Goldstein LH, Abrahams S. Changes in cognition and behaviour in amyotrophic lateral sclerosis: nature of impairment and implications for assessment. Lancet Neurol 2013; 12 (4): 368–380. doi: 10.1016/S1474-4422 (13) 70 026-7.

100. Beeldman E, Raaphorst J, Klein Twenaar M et al. The cognitive profile of behavioural variant FTD and its similarities with ALS: a systematic review and meta-analysis. J Neurol Neurosurg Psychiatry 2018; 89 (9): 995–1002. doi: 10.1136/jnnp-2017-317459.

101. Yunusova Y, Ansari J, Ramirez J et al. Frontal anatomical correlates of cognitive and speech motor deficits in amyotrophic lateral sclerosis. Behav Neurol 2019; 2019: 9518309. doi: 10.1155/2019/9518309.

102. Leslie FV, Hsieh S, Caga J et al. Semantic deficits in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener 2015; 16 (1–2): 46–53. doi: 10.3109/21678421.2014.987301.

103. Kamminga J, Leslie FV, Hsieh S et al. Syntactic comprehension deficits across the FTD-ALS continuum. Neurobiol Aging 2016; 41: 11–18. doi: 10.1016/j.neurobiolaging.2016.02.002.

104. Bezdíček O, Marková L, Soósová N et al. Pilotní studie Edinburské kognitivně-behaviorální zkoušky pro amyotrofickou laterální sklerózu na české populaci. Cesk Slov Neurol N 2018; 81 (2): 208–212. doi: 10.14735/amcsnn2018208.

105. Rusina R., Matěj R. Frontotemporální lobární degenerace. In: Rusina R, Matěj R, Bartoš A et al. Neurodegenerativní onemocnění. 2. vyd. Praha: Mladá fronta 2019: 177–191.

106. Forsberg K, Graffmo K, Pakkenberg B et al. Misfolded SOD1 inclusions in patients with mutations in C9orf72 and other ALS/FTD-associated genes. J Neurol Neurosurg Psychiatry 2019; 90 (8): 861–869. doi: 10.1136/jnnp-2018-319386.

107. Siddique N, Siddique T. Amyotrophic lateral sclerosis overview. [online]. Available from: https: //www.ncbi.nlm.nih.gov/books/NBK1450/.

108. Geser F, Lee VM, Trojanowski JQ. Amyotrophic lateral sclerosis and frontotemporal lobar degeneration: a spectrum of TDP-43 proteinopathies. Neuropathology 2010; 30 (2): 103–112. doi: 10.1111/j.1440-1789.2009.01091.x

109. Harrison KL, Hunt LJ, Ritchie CS et al. Dying with dementia: Underrecognized and stigmatized. J Am Geriatr Soc 2019; 67 (8): 1548–1551. doi: 10.1111/jgs.15895.

110. Rusina R, Špeciánová Š. Paliativní aspekty u pokročilé demence. Neurol praxi 2020; 21 (2): 100–102. doi: 10.36290/neu.2020.056.

111. Musa G, Slachevsky A, Muñoz-Neira C et al. Alzheimer‘s disease or behavioral variant frontotemporal dementia? Review of key points toward an accurate clinical and neuropsychological diagnosis. J Alzheimers Dis 2020; 73 (3): 833–848. doi: 10.3233/JAD-190924.

112. Li Y, Hai S, Zhou Y et al. Cholinesterase inhibitors for rarer dementias associated with neurological conditions. Cochrane Database Syst Rev 2015; (3): CD009444. doi: 10.1002/14651858.CD009444.pub3.

113. Peters ME, Schwartz S, Han D et al. Neuropsychiatric symptoms as predictors of progression to severe Alzheimer’s dementia and death: the Cache County Dementia Progression Study. Am J Psychiatry 2015; 172 (5): 460–465. doi: 10.1176/appi.ajp.2014.14040480.

114. Dubois B, Feldman H, Jacova C et al. Advancing research diagnostic criteria for Alzheimer’s disease: the IWG-2 criteria. Lancet Neurol 2014; 13 (6): 614–629. doi: 10.1016/S1474-4422 (14) 70090-0.

115. Johnson JK, Head E, Kim R et al. Clinical and pathological evidence for a frontal variant of Alzheimer disease. Arch Neurol 1999; 56 (10): 1233–1239. doi: 10.1001/archneur.56.10.1233.

116. Ossenkoppele R, Pijnenburg YA, Perry DC et al. The behavioural/dysexecutive variant of Alzheimer’s disease: clinical, neuroimaging and pathological features. Brain 2015; 138 (Pt 9): 2732–2749. doi: 10.1093/brain/ awv191.

117. Matušková V, Nikolai T; Marková H et al. Neuropsychiatrické symptomy jako časná manifestace Alzheimerovy nemoci. Cesk Slov Neurol N 2020; 83 (1): 64–72. doi: 10.14735/amcsnn202064.

118. Sawyer RP, Rodriguez-Porcel F, Hagen M et al. Diagnosing the frontal variant of Alzheimer‘s disease: a clinician‘s yellow brick road. J Clin Mov Disord 2017; 4: 2. doi: 10.1186/s40734-017-0052-4.

119. Alladi S, Xuereb J, Bak T et al. Focal cortical presentations of Alzheimer’s disease. Brain 2007; 130 (Pt 10): 2636–2645. doi: 10.1093/brain/awm213.

120. Leger GC, Banks SJ. Neuropsychiatric symptom profile differs based on pathology in patients with clinically diagnosed behavioral variant frontotemporal dementia. Dement Geriatr Cogn Disord 2014; 37 (1–2): 104–112. doi: 10.1159/000354368.

121. Bathgate D, Snowden JS, Varma A et al. Behaviour in frontotemporal dementia, Alzheimer’s disease and vascular dementia. Acta Neurol Scand 2001; 103 (6): 367–378. doi: 10.1034/j.1600-0404.2001.2000236.x

122. Bozeat S, Gregory CA, Ralph MA et al. Which neuropsychiatric and behavioural features distinguish frontal and temporal variants of frontotemporal dementia from Alzheimer’s disease? J Neurol Neurosurg Psychiatry 2000; 69 (2): 178–186. doi: 10.1136/jnnp.69.2.178.

123. Mendez MF, Shapira JS, Miller BL. Stereotypical movements and frontotemporal dementia. Mov Disord 2005; 20 (6): 742–745. doi: 10.1002/mds.20465.

124. Rascovsky K, Salmon DP, Ho GJ et al. Cognitive profiles differ in autopsy-confirmed frontotemporal dementia and AD. Neurology 2002; 58 (12): 1801–1808. doi: 10.1212/wnl.58.12.1801.

125. Libon DJ, McMillan C, Gunawardena D et al. Neurocognitive contributions to verbal fluency deficits in frontotemporal lobar degeneration. Neurology 2009; 73 (7): 535–542. doi: 10.1212/WNL.0b013e3181b2a4f5.

126. Hutchinson AD, Mathias JL. Neuropsychological deficits in frontotemporal dementia and Alzheimer’s disease: a meta-analytic review. J Neurol Neurosurg Psychiatry 2007; 8 (9): 917–928. doi: 10.1136/jnnp.2006.100669.

127. Elfgren C, Brun A, Gustafson L et al. Neuropsychological tests as discriminators between dementia of Alzheimer type and frontotemporal dementia. Int J Geriatric Psychiatry 1994; 9 (8): 635–642.

128. Lindau M, Almkvist O, Johansson SE et al. Cognitive and behavioral differentiation of frontal lobe degeneration of the non-Alzheimer type and Alzheimer‘s disease. Dement Geriatr Cogn Disord 1998; 9 (4): 205–213. doi: 10.1159/000017048.

129. Giovagnoli AR, Erbetta A, Reati F et al. Differential neuropsychological patterns of frontal variant frontotemporal dementia and Alzheimer‘s disease in a study of diagnostic concordance. Neuropsychologia 2008; 46 (5): 1495–1504. doi: 10.1016/j.neuropsychologia.2007.12.023.

130. Barber R, Snowden JS, Craufurd D. Frontotemporal dementia and Alzheimer’s disease: retrospective differentiation using information from informants. J Neurol Neurosurg Psychiatry 1995; 59 (1): 61–70. doi: 10.1136/jnnp.59.1.61.

131. Mendez MF, McMurtray AM, Licht EA et al. Frontal-executive versus posterior-perceptual mental status deficits in early-onset dementias. Am J Alzheimers Dis Other Demen 2009; 24 (3): 220–227. doi: 10.1177/1533317509332626.

132. Baum, LF. Čaroděj ze země Oz / The Wonderful Wizard of Oz. Praha: Garamond 2018: 308.

133. Andrews SJ, McFall GP, Booth A et al. Association of Alzheimer‘s disease genetic risk loci with cognitive performance and decline: a systematic review. J Alzheimers Dis 2019; 69 (4): 1109–1136. doi: 10.3233/JAD-190342.

134. DeTure MA, Dickson DW. The neuropathological diagnosis of Alzheimer‘s disease. Mol Neurodegener 2019; 14 (1): 32. doi: 10.1186/s13024-019-0333-5.

135. Montine TJ, Phelps CH, Beach TG et al. National Institute on Aging-Alzheimer‘s Association guidelines for the neuropathologic assessment of Alzheimer‘s disease: a practical approach. Acta Neuropathol 2012; 123 (1): 1–11. doi: 10.1007/s00401-011-0910-3.

136. Greenberg SM, Bacskai BJ, Hernandez-Guillamon M et al. Cerebral amyloid angiopathy and Alzheimer disease – one peptide, two pathways. Nat Rev Neurol 2020; 16 (1): 30–42. doi: 10.1038/s41582-019-0281-2.

137. Miller BL. Frontotemporal dementia. Oxford: Oxford University Press 2013: 32–35.

138. Devenney E, Swinn T, Mioshi E et al. The behavioural variant frontotemporal dementia phenocopy syndrome is a distinct entity – evidence from a longitudinal study. BMC Neurol 2018; 18 (1): 56. doi: 10.1186/s12883-018-1060-1.

139. Valente ES, Caramelli P, Gambogi LB et al. Phenocopy syndrome of behavioral variant frontotemporal dementia: a systematic review. Alzheimers Res Ther 2019; 11 (1): 30. doi: 10.1186/s13195-019-0483-2.

140. Mezinárodní klasifikace nemocí. 10. revize. Duševní poruchy a poruchy chování. Popisy klinických příznaků a diagnostická vodítka. Praha: Psychiatrické centrum 1992.