Issues of diagnosis and treatment of frontotemporal degeneration
https://doi.org/10.37586/2686-8636-3-2020-217-224
Abstract
Frontotemporal degeneration (FTD) is a heterogeneous group of progressive neurodegenerative diseases associated with a predominant lesion of the frontal and/or anterior temporal lobes of the brain. FTD is at the fourth place in the list of causes of severe neurocognitive disorders in all age groups and at the second place among patients of presenile age (up to 65 years). FTD is a genetically determined disease. In 30-40%, there is a family history, which is characterized by an autosomal dominant type of transmission in up to 15% of cases. Usually FTD begins in the fifth or sixth decade of life, but a later onset is possible. The article discusses the main clinical variants of FTD. The behavioral variant of FTD accounts for more than half of the cases of FTD and is characterized by a combination of cognitive, behavioral, and emotional-affective symptoms, atrophy of the frontal and temporal lobes of the brain. In primary progressive aphasia (PPA), speech disorders are the most significant clinical symptoms for at least two years. The clinical features of PPA depend on the localization of the pathological process. The division into clinical variants of FTD is relevant only in the first years of the disease. In the future, the differences between them are erased, and the status may contain several clinical variants simultaneously. In the treatment of FTD, a comprehensive approach is presented, including symptomatic therapy, as well as non-drug treatments (educational program, psychological support, cognitive training, speech therapy). Accurate analysis of emotional and behavioral symptoms in FTD can help predict the course of the pathological process, optimize symptomatic treatment, and there by improve the quality of life of patients and their relatives.
About the Author
D. A. Grishina
Federal State Autonomous Educational Institution of Higher Education I.M. Sechenov First Moscow State Medical University of the Ministry of Healthcare of the Russian Federation (Sechenovskiy University); Pirogov Russian National Research Medical University of Ministry of Health of the Russian Federation, Russian Gerontology Research and Clinical Centre
Russian Federation
Russian Federation
MD, PhD, Assistant of the Department of Nervous Diseases and Neurosurgery Faculty of General Medicine, Federal State Autonomous Educational Institution of Higher Education Sechenov First Moscow State Medical University of the Ministry of Heath of the Russian Federation (Sechenov University); neurologist, Pirogov National Research Medical University, Russian Gerontology Research and Clinical Centre.
Moscow.
+7(985) 304-72-39
References
1. Rascovsky K., Hodges J.R., Knopman D., Mendez M.F., Kramer J.H., Neuhaus J., van Swieten J.C., Seelaar H., Dopper E.G., Onyike C.U., Hillis A.E., Josephs K.A., Boeve B.F., Kertesz A., Seeley W.W., Rankin K.P., Johnson J.K., Gorno-Tempini M.L., Rosen H., Prioleau-Latham C.E., Lee A., Kipps C.M., Lillo P., Piguet O., Rohrer J.D., Rossor M.N., Warren J.D., Fox N.C., Galasko D., Salmon D.P., Black S.E., Mesulam M., Weintraub S., Dickerson B.C., Diehl-Schmid J., Pasquier F., Deramecourt V., Lebert F., Pijnenburg Y., Chow T.W., Manes F., Grafman J., Cappa S.F., Freedman M., Grossman M., Miller B.L. Sensitivity ofrevised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain. 2011; 134 (Pt 9): 2456-77. DOI: 10.1093/brain/awr179.
2. Grishina D.A., Zakharov V.V., Yakhno N.N. Cognitive disorders in behavioral variant of frontotemporal dementia. Nevrologicheskiy Zhurnal. 2016; 21(6): 330-337 (in Russ.). DOI: http://dx.doi.org/10.18821/1560-9545-2016-21-6-330-337.
3. Grishina D.A., Yakhno N.N., Zakharov V.V. Emotional, affective and behavioral disorders in a behavioral variant of frontotemporal dementia. Zhurnal nevrologii, psikhiatrii im S.S. Korsakova. 2017; 117(11): 13-17. (in Russ.) DOI: 10.17116/jnevro201711711113-17.
4. Yakhno N.N., Zakharov V.V., Lokshina A.B., Koberskaya N.N., Mkhitaryan E.A. Dementia. Guidance for doctors. Moscow: MEDpressinform; 2010. (in Russ.). https://www.03book.ru/upload/iblock/987/415_Demencija_Jahno.pdf.
5. Seelaar H., Rohrer J.D., Pijnenburg Y.A., Fox N.C., van Swieten J.C. Clinical, genetic and pathological heterogeneity of frontotemporal dementia: a review. J Neurol Neurosurg Psychiatry. 2011; 82(5): 476-86. DOI: 10.1136/jnnp.2010.212225.
6. Onyike C.U., Diehl-Schmid J. The epidemiology of frontotemporal dementia. Int Rev Psychiatry. 2013; 25(2): 130-7. DOI: 10.3109/09540261.2013.776523.
7. Coyle-Gilchrist I.T., Dick K.M., Patterson K., Vazquez Rodriquez P., Wehmann E., Wilcox A., Lansdall C.J., Dawson K.E., Wiggins J., Mead S., Brayne C., Rowe J.B. Prevalence, characteristics, and survival of frontotemporal lobar degeneration syndromes. Neurology 2016; 86(18): 1736-1743. DOI: 10.1212/WNL.0000000000002638.
8. Goldman J.S., Farmer J.M., Wood E.M., Johnson J.K., Boxer A., Neuhaus J., Lomen-Hoerth C., Wilhelmsen K.C., Lee V.M., Grossman M., Miller B.L. Comparison of family histories in FTLD subtypes and related tauopathies. Neurology. 2005; 65(11): 1817-9. DOI: 0.1212/01.wnl.0000187068.92184.63.
9. Greaves C.V., Rohrer J.D. An update on genetic frontotemporal dementia. J Neurol. 2019; 266(8): 2075-2086. DOI: 10.1007/s00415-019-09363-4.
10. Mackenzie I.R., Neumann M. Molecular neuropathology of frontotemporal dementia: insights into disease mechanisms from postmortem studies. J Neurochem. 2016 Aug; 138 Suppl 1: 54-70. DOI: 10.1111/jnc.13588.
11. Stepkina D.A., Zakharov V.V., Yakhno N.N. Primary progressive aphasia syndrome. Nevrologicheskiy Zhurnal.2014; 5: 22-28. (in Russ.) https://cyberleninka.ru/article/n/sindrom-pervichnoy-progressiruyuschey-afazii.
12. Gorno-Tempini M.L., Hillis A.E., Weintraub S., Kertesz A., Mendez M., Cappa S.F., Ogar J.M, Rohrer J.D., Black S., Boeve B.F., Manes F., Dronkers N.F., Vandenberghe R., Rascovsky K., Patterson K., Miller B.L., Knopman D.S., Hodges J.R., Mesulam M.M., Grossman M. Classification of primary progressive aphasia and its variants. Neurology. 2011; 76(11): 1006-14. DOI: 10.1212/WNL.0b013e31821103e6.
13. Cosseddu M., Benussi A., Gazzina S., Alberici A., Dell'Era V., Manes M., Cristillo V., Borroni B, Padovani A. Progression of behavioural disturbances in frontotemporal dementia: A longitudinal observational study. Eur J Neurol. 2020; 27(2): 265272. DOI: 10.1111/ene.14071.
14. Liljegren M., Naasan G., Temlett J., Perry D.C., Rankin K.P., Merrilees J., Miller B.L. Criminal behavior in frontotemporal dementia and Alzheimer disease. JAMA Neurol. 2015; 72(3): 295-300. DOI: 10.1001/jamaneurol.2014.3781.
15. Rosen H.J., Allison S.C, Schauer G.F., Gorno-Tempini M.L., Weiner M.W., Miller B.L. Neuro-anatomical correlates of behavioural disorders in dementia. Brain. 2005; 128(Pt 11): 2612-25. DOI: 10.1093/brain/awh628.
16. Woolley J.D., Gorno-Tempini M.L., Seeley W.W., Rankin K., Lee S.S., Matthews B.R., Miller B.L. Binge eating is associated with right orbi-tofrontal-insular-striatal atrophy in frontotemporal dementia. Neurology. 2007; 69(14): 1424-33. DOI: 10.1212/01.wnl.0000277461.06713.23.
17. Blass D.M., Rabins P.V. Depression in frontotemporal dementia. Psychosomatics. 2009; 50(3): 239-247. DOI: 10.1176/appi.psy.50.3.239.
18. Mendez M.F., McMurtray A., Chen A.K., Shapira J. S., Mishkin F., Miller B. L. Functional neuroimaging and presenting psychiatric features in frontotemporal dementia. Neurol Neurosurg Psychiatry. 2006; 77(1): 4-7. DOI: 10.1136/jnnp.2005.072496.
19. Cummings J.L., Mega M., Gray K., Rosenberg-Thompson S., Carusi D.A., Gornbein J. The Neuropsychiatric Inventory: comprehensive assessment of psychopathology in dementia. Neurology. 1994; 44(12): 2308-14. DOI: 10.1212/wnl.44.12.2308.
20. Kertesz A., Nadkarni N., Davidson W., Thomas A.W. Frontal behavioral inventory: diagnostic criteria for frontal lobe dementia Can J Neurol Sci. 1997; 24(1): 29-36. DOI: 10.1017/s0317167100021053.
21. Torralva T., Roca M., Gleichgerrcht E., Bekinschtein T., Manes F. A neuropsychological battery to detect specific executive and social cognitive impairments in early frontotemporal dementia. Brain. 2009; 132(Pt5): 1299-309. DOI: 10.1093/brain/awp041.
22. Hornberger M., Piguet O., Graham A.J., Nestor P.J., Hodges J.R. How preserved is episodic memory in behavioral variant frontotemporal dementia? Neurology. 2010; 74 (6): 472-9. DOI: 10.1212/WNL.0b013e3181cef85d.
23. Hardy C.J., Buckley A.H., Downey L.E., Lehmann M., Zimmerer V.C., Varley R.A., Crutch S.J., Rohrer J.D., Warrington E.K., Warren J.D. The language profile of behavioral variant frontotemporal dementia. J Alzheimers Dis. 2016; 50(2): 359-71. DOI: 10.3233/JAD-150806.
24. Grossman M. Primary progressive aphasia: clinicopathological correlations. Nat Rev Neurol. 2010; 6(2): 88-97. DOI: 10.1038/nrneurol.2009.216.
25. Whitwell J.L., Boeve B.F., Weigand S.D., Senjem M.L, Gunter J.L., Baker M.C., DeJesus-Hernandez M., Knopman D.S., Wszolek Z.K., Petersen R.C., Rademakers R., Jack C.R. Jr., Josephs K.A. Brain atrophy over time in genetic and sporadic frontotemporal dementia: a study of 198 serial magneticresonance images. Eur J Neurol. 2015; 22(5): 745-752. DOI: 10.1111/ene.12675.
26. Stepkina D.A., Zakharov V.V., Yakhno N.N. Non-cognitive neuropsychiatric disorders in patients with primary progressive aphasia syndrome. Nevrologicheskiy Zhurnal. 2014; 19 (6): 17-22. (in Russ.) https://cyberleninka.ru/article/n/nekognitivnye-nervno-psihicheskie-narusheniya-pri-sindrome-pervichnoy-progressiruyuschey-afazii
27. Woolley J.D., Khan B.K., Murthy N.K., Miller B.L., Rankin K.P. 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 Feb; 72(2): 126-133. DOI: 10.4088/JCP.10m06382oli.
28. Velakoulis D., Walterfang M., Mocellin R., Pantelis C., McLean C. Frontotemporal dementia presenting as schizophrenia-like psychosis in young people: clinicopathological series and review of cases. Br J Psychiatry. 2009; 194 (4): 298-305. DOI: https://doi.org/10.1192/bjp.bp.108.057034
29. Gorno-Tempini M.L., Brambati S.M., Ginex V. Ogar J., Dronkers N.F., Marcone A., Perani D., Garibotto V., Cappa S.F., Miller B.L. The logopenic/ phonological variant of primary progressive aphasia. Neurology. 2008; 71(16): 1227-1234. DOI:10.1212/01.wnl.0000320506.79811.da
30. Ewers M., Mattsson N., Minthon L. Molinuevo J.L., Antonell A., Popp J., Jessen F., Herukka S.K., Soininen H., Maetzler W., Leyhe T., Burger K., Taniguchi M., Urakami K., Lista S., Dubois B., Blennow K., Hampel H. CSF biomarkers for the differential diagnosis of Alzheimer's disease: A large-scale international multicenter study. Alzheimers Dement. 2015; 11(11): 1306-15. DOI: 10.1016/j.jalz.2014.12.006.
31. Kortte K.B., Rogalski E.J. Behavioural interventions for enhancing life participation in behavioural variant frontotemporal dementia and primary progressive aphasia. Int. Rev. Psychiatry. 2013; 25(2): 237-45. DOI: 10.3109/09540261.2012.751017.
32. Cheng S.T., Chow P.K., Song Y.Q., Yu E.C., Chan A.C., Lee T.M., Lam J.H. Mental and physical activities delay cognitive decline in older persons with dementia. Am J Geriatr Psychiatry. 2014; 22(1): 6374. DOI: 10.1016/j.jagp.2013.01.060.
33. Mendez M.F., Shapira J.S., McMurtray A., Licht E. Preliminary findings: behavioral worsening on donepezil in patients with frontotemporal dementia. Am. J. Geriatr. Psychiatry. 2007; 15: 84-87. DOI: 10.1097/01.JGP.0000231744.69631.33.
34. Kertesz A., Morlog D., Light M., Blair M., Davidson W., Jesso S., Brashear R. Galantamine in frontotemporal dementia and primary progressive aphasia Dement Geriatr Cogn Disord. 2008; 25(2): 178-85. DOI: 10.1159/000113034.
35. Kishi T., Matsunaga S., Iwata N. Memantine for the treatment of frontotemporal dementia: a metaanalysis. Neuropsychiatr Dis Treat. 2015; 11: 2883-5. DOI: 10.2147/NDT.S94430. eCollection 2015.
36. Boxer A.L., Lipton A.M., Womack K., Merrilees J., Neuhaus J., Pavlic D., Gandhi A., Red D., Martin-Cook K., Svetlik D., Miller B.L. An openlabel study of Memantine treatment in 3 subtypes of frontotemporal lobar degeneration. Alzheimer Dis Assoc Disord. 2009. 23(3): 211-7. DOI: 10.1097/WAD.0b013e318197852f.
37. Johnson N.A., Rademaker A., Weintraub S., Gitelman D., Wienecke C., Mesulam M. Pilot trail of memantine in primary progressive aphasia. Alzheimer Dis Assoc Disord. 2010; 24(3): 308. DOI: 10.1097/WAD.0b013e3181cf468d.
38. Procter A.W., Qurne M., Francis P.T. Neurochemical features of fronto-temporal dementia. Dement Geriatr Cogn Disord. 1999; 10 Suppl 1: 80-4. DOI: 10.1159/000051219.
39. Franceschi M., Anchisi D., Pelati O., Zuffi M., Matarrese M., Moresco R.M., Fazio F., Perani D. Glucose metabolism and serotonin receptors in the frontotemporal lobe degeneration. Ann. Neurol. 2005; 57: 216-225. DOI: 10.1002/ana.20365.
40. Herrmann N., Black S.E., Chow T., Cappell J., Tang-Wai D.F., Lanctot K.L. Serotonergic function and treatment of behavioral and psychological symptoms of frontotemporal dementia. Am J Geriatr Psychiatry. 2012; 20(9): 789-97. DOI: 10.1097/JGP.0b013e31823033f3.
41. Pijnenburg Y.A., Sampson E.L., Harvey R.J., Fox N.C., Rossor M.N. Vulnerability to neuroleptic side effects in frontotemporal lobar degeneration. Int J Geriatr Psychiatry. 2003; 18(1): 67-72. DOI: 10.1002/gps.774.
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For citations:
Grishina D.A. Issues of diagnosis and treatment of frontotemporal degeneration. Russian Journal of Geriatric Medicine. 2020;(3):217-224. (In Russ.) https://doi.org/10.37586/2686-8636-3-2020-217-224
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