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DOI: 10.1590/0004-282X-ANP-2022.S140
How to differentiate behavioral variant frontotemporal dementia from primary psychiatric disorders: practical aspects for the clinician
Como diferenciar variante comportamental da demência frontotemporal de transtornos psiquiátricos: aspectos práticos para o clínicoABSTRACT
Background: Due to the early and prominent behavioral changes which characterize behavioral variant frontotemporal dementia (bvFTD), patients are more likely to seek psychiatric help and are often initially diagnosed with a primary psychiatric disorder (PPD). Differentiating these conditions is critical because of the dramatically different outcomes, differences in patient management, family counseling and caregiver education. Objective: To propose a practical guide to distinguish between bvFTD and PDD. Methods: We conducted a non-systematic review of the published manuscripts in the field, including some previous investigations from our own group and work on which we have collaborated, and summarized the main findings and proposals that may be useful for neurological practice. Results: The reviewed literature suggests that a comprehensive clinical history, brief cognitive and neuropsychological evaluations, detailed neurological examination with special attention to motor alterations related to bvFTD, structural and functional neuroimaging evaluation, genetic investigation in selected cases, and assistance from a multidisciplinary team, including a neurologist and a psychiatrist with expertise in bvFTD, are very helpful in differentiating these conditions. Conclusions: Although the clinician may commonly face great difficulty in differentiating between bvFTD and PPD, the use of appropriate tools in a systematic way and the availability of a well-trained multidisciplinary group can significantly increase diagnostic accuracy.
RESUMO
Antecedentes: Devido às alterações comportamentais precoces e proeminentes que caracterizam a variante comportamental da demência frontotemporal (DFTvc), os pacientes são mais propensos a procurar atendimento psiquiátrico e muitas vezes são diagnosticados inicialmente com um transtorno psiquiátrico primário (TPP). Diferenciar essas condições é fundamental, devido aos desfechos significativamente diferentes, diferenças no manejo dos pacientes, no aconselhamento familiar e na educação dos cuidadores. Objetivo: Propor um guia prático para o diagnóstico diferencial entre DFTvc e TPP. Métodos: Revisão não sistemática dos manuscritos publicados na área, incluindo algumas investigações anteriores de nosso próprio grupo e trabalhos com os quais colaboramos. Os principais achados e propostas foram sintetizados para auxiliarem a prática neurológica. Resultados: A literatura revisada sugere que a história clínica abrangente, avaliação cognitiva breve e avaliação neuropsicológica, exame neurológico detalhado com especial atenção às alterações motoras relacionadas à DFTvc, exames de neuroimagem estrutural e funcional, investigação genética em casos selecionados e o atendimento por equipe multidisciplinar, incluindo um neurologista e um psiquiatra com experiência em DFTvc, são bastante úteis na diferenciação dessas condições. Conclusões: Embora o clínico possa enfrentar grande dificuldade para diferenciar DFTvc e TPP, o uso de ferramentas apropriadas de forma sistemática e a disponibilidade de uma equipe multidisciplinar bem treinada podem aumentar significativamente a acurácia diagnóstica.
Authors’ contributions:
LBG: conceptualization, writing and editing; LCS: manuscript review; PC: conceptualization, writing, and final editing.
Publication History
Received: 13 April 2022
Accepted: 29 April 2022
Article published online:
06 February 2023
© 2022. Academia Brasileira de Neurologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)
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References
- 1 Ljubenkov PA, Miller BL.. A clinical guide to frontotemporal dementias. Focus (Am Psychiatr Publ) 2016; 14 (04) 448-464 https://doi.org/10.1176/appi.focus.20160018
- 2 Lanata SC, Miller BL.. The behavioural variant frontotemporal dementia (bvFTD) syndrome in psychiatry. J Neurol Neurosurg Psychiatry 2016; 87 (05) 501-511 https://doi.org/10.1136/jnnp-2015-310697
- 3 Woolley JD, Khan BK, Murthy NK, Miller BL, Rankin KP. 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 (02) 126-133 https://doi.org/10.4088/jcp.10m06382oli
- 4 Chow TW, Pio FJ, Rockwood K.. An international needs assessment of caregivers for frontotemporal dementia. Can J Neurol Sci 2011; 38 (05) 753-757 https://doi.org/10.1017/s0317167100054147
- 5 Rascovsky K, Hodges JR, Knopman D, Mendez MF, Kramer JH, Neuhaus J. et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain 2011; 134 (09) 2456-2477 https://doi.org/10.1093/brain/awr179
- 6 Olney NT, Spina S, Miller BL.. Frontotemporal dementia. Neurol Clin 2017; 35 (02) 339-374 https://doi.org/10.1016/j.ncl.2017.01.008
- 7 Bang J, Spina S, Miller BL.. Frontotemporal dementia. Lancet 2015; 386 10004 1672-1682 https://doi.org/10.1016/s0140-6736(15)00461-4
- 8 Onyike CU, Diehl-Schmid J.. The epidemiology of frontotemporal dementia.. Int Rev Psychiatry 2013; (02) 130-137
- 9 Llibre-Guerra JJ, Behrens MI, Hosogi ML, Montero L, Torralva T, Custodio N. et al. Frontotemporal dementias in Latin America: History, epidemiology, genetics, and clinical research. Front Neurol. 2021; Sep; (12) 710332 https://doi.org/10.3389/fneur.2021.710332
- 10 Ducharme S, Price BH, Larvie M, Dougherty DD, Dickerson BC.. Clinical approach to the differential diagnosis between behavioral variant frontotemporal dementia and primary psychiatric disorders. Am J Psychiatry 2015; Sep; 172 (09) 827-837 https://doi.org/10.1176/appi.ajp.2015.14101248
- 11 Dols A, Krudop W, Moller C, Shulman K, Sajatovic M, Pijnenburg YA.. Late life bipolar disorder evolving into frontotemporal dementia mimic. Neuropsychiatr Dis Treat 2016; Sep; 12: 2207-2212 https://doi.org/10.2147/ndt.s99229
- 12 Gossink FT, Dols A, Krudop WA, Sikkes SA, Kerssens CJ, Prins ND. et al. Formal psychiatric disorders are not overrepresented in behavioral variant frontotemporal dementia. J Alzheimers Dis 2016; 51 (04) 1249-1256 https://doi.org/10.3233/jad-151198
- 13 Cardarelli R, Kertesz A, Knebl JA.. Frontotemporal dementia: A review for primary care physicians. Am Fam Physician 2010; Dec; 82 (11) 1372-1377
- 14 Gregory CA.. Frontal variant of frontotemporal dementia: A cross-sectional and longitudinal study of neuropsychiatric features.. Psychol Med 1999; 29 (05) 1205-1217 https://doi.org/10.1017/s0033291799008934
- 15 Levy R, Dubois B.. Apathy and the functional anatomy of the prefrontal cortex-basal ganglia circuits. Cereb Cortex 2006; 16 (07) 916-928 https://doi.org/10.1093/cercor/bhj043
- 16 Woolley JD, Wilson MR, Hung E, Gorno-Tempini ML, Miller BL, Shim J.. Frontotemporal dementia and mania. Am J Psychiatry 2007; 164 (12) 1811-1816 https://doi.org/10.1176/appi.ajp.2007.07061001
- 17 Perry RJ, Miller BL.. Behavior and treatment in frontotemporal dementia. Neurology 2001; Jun;56(11 Suppl 4): S46-S51 https://doi.org/10.1212/wnl.56.suppl_4.s46
- 18 Mann-Wrobel MC, Carreno JT, Dickinson D.. Meta-analysis of neuropsychological functioning in euthymic bipolar disorder: an update and investigation of moderator variables. Bipolar Disord 2011; 13 (04) 334-342 https://doi.org/10.1111/j.1399-5618.2011.00935.x
- 19 Gambogi LB, Guimaraes HC, de Souza LC, Caramelli P.. Long-Term severe mental disorders preceding behavioral variant frontotemporal dementia: Frequency and clinical correlates in an outpatient sample. J Alzheimers Dis 2018; 66 (04) 1577-1585 https://doi.org/10.3233/jad-180528
- 20 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 (04) 298-305 https://doi.org/10.1192/bjp.bp.108.057034
- 21 Devenney EM, Landin-Romero R, Irish M, Hornberger M, Mioshi E, Halliday GM. et al. The neural correlates and clinical characteristics of psychosis in the frontotemporal dementia continuum and the C9orf72 expansion. Neuroimage Clin 2017; 13: 439-445 https://doi.org/10.1016/j.nicl.2016.11.028
- 22 Bleuler E.. Dementia praecox: Oder Gruppe der Schizophrenien. Leipzig: Deuticke; 1911. 420.
- 23 Carpenter Jr WT, Strauss JS, Bartko JJ. An approach to the diagnosis and understanding of schizophrenia. Introduction. Schizophr Bull 1974; 1 (11) 35-36 https://doi.org/10.1093/schbul/1.11.35
- 24 Zipursky RB, Reilly TJ, Murray RM.. The myth of schizophrenia as a progressive brain disease.. Schizophr Bull 2013; 39 (06) 1363-1372 https://doi.org/10.1093/schbul/sbs135
- 25 Ducharme S, Dickerson BC, Larvie M, Price BH.. Differentiating frontotemporal dementia from catatonia: A complex neuropsychiatric challenge. J Neuropsychiatry Clin Neurosci 2015; 27 (02) e174-6 https://doi.org/10.1176/appi.neuropsych.14070157
- 26 Freitas S, Simoes MR, Alves L, Duro D, Santana I.. Montreal Cognitive Assessment (MoCA): Validation study for frontotemporal dementia. J Geriatr Psychiatry Neurol 2012; 25 (03) 146-154 https://doi.org/10.1177/0891988712455235
- 27 Krudop WA, Kerssens CJ, Dols A, Prins ND, Möller C, Schouws S. et al. Identifying bvFTD within the wide spectrum of late onset frontal lobe syndrome: A clinical approach. Am J Geriatr Psychiatry 2015; 23 (10) 1056-1066 https://doi.org/10.1016/j.jagp.2015.04.002
- 28 Fiorentino N, Gleichgerrcht E, Roca M, Cetkovich M, Manes F, Torralva T.. The INECO Frontal Screening tool differentiates behavioral variant - frontotemporal dementia (bv-FTD) from major depression. Dement Neuropsychol 2013; 7 (01) 33-39 https://doi.org/10.1590/s1980-57642013dn70100006
- 29 Amaral-Carvalho V, Lima-Silva TB, Mariano LI, de Souza LC, Guimarães HC, Bahia VS. et al. Brazilian version of Addenbrooke's Cognitive Examination-Revised in the differential diagnosis of Alzheimer's disease and behavioral variant frontotemporal dementia. Arch Clin Neuropsychol 2022; Feb; 37 (02) 437-438 https://doi.org/10.1093/arclin/acab071
- 30 Dodich A, Crespi C, Santi GC, Cappa SF, Cerami C.. Evaluation of discriminative detection abilities of social cognition measures for the diagnosis of the behavioral variant of frontotemporal dementia: A systematic review. Neuropsychol Rev 2021; 31 (02) 251-266 https://doi.org/10.1007/s11065-020-09457-1
- 31 Bertoux M, Volle E, de Souza LC, Funkiewiez A, Dubois B, Habert MO. Neural correlates of the mini-SEA (Social cognition and Emotional Assessment) in behavioral variant frontotemporal dementia. Brain Imaging Behav 2014; 8 (01) 1-6 https://doi.org/10.1007/s11682-013-9261-0
- 32 Mariano LI, Caramelli P, Guimaraes HC, Gambogi LB, Moura MVB, Yassuda MS. et al. Can social cognition measurements differentiate behavioral variant frontotemporal dementia from Alzheimer's disease regardless of apathy?. J Alzheimers Dis 2020; 74 (03) 817-827 https://doi.org/10.3233/jad-190861
- 33 Bertoux M, Delavest M, de Souza LC, Funkiewiez A, Lepine JP, Fossati P. et al. Social cognition and emotional assessment differentiates frontotemporal dementia from depression. J Neurol Neurosurg Psychiatry 2012; 83 (04) 411-416 https://doi.org/10.1136/jnnp-2011-301849
- 34 Chiu I, Piguet O, Diehl-Schmid J, Riedl L, Beck J, Leyhe T. et al. Facial emotion recognition performance differentiates between behavioral variant frontotemporal dementia and major depressive disorder. J Clin Psychiatry 2018; 79 (01) 16m11324 https://doi.org/10.4088/jcp.16m11342
- 35 Baez S, Pinasco C, Roca M, Ferrari J, Couto B, García-Cordero I. et al. Brain structural correlates of executive and social cognition profiles in behavioral variant frontotemporal dementia and elderly bipolar disorder. Neuropsychologia 2019; 126: 159-169 https://doi.org/10.1016/j.neuropsychologia.2017.02.012
- 36 Filippi M, Agosta F, Barkhof F, Dubois B, Fox NC, Frisoni GB. et al. EFNS task force: The use of neuroimaging in the diagnosis of dementia. Eur J Neurol 2012; 19 (12) 1487-1501 https://doi.org/10.1111/j.1468-1331.2012.03859.x
- 37 Soucy JP, Bartha R, Bocti C, Borrie M, Burhan AM, Laforce R. et al. Clinical applications of neuroimaging in patients with Alzheimer's disease: A review from the Fourth Canadian Consensus Conference on the Diagnosis and Treatment of Dementia 2012. Alzheimers Res Ther 2013; 5 (Suppl. 01) S3 https://doi.org/10.1186/alzrt199
- 38 Peet BT, Spina S, Mundada N, La Joie R.. Neuroimaging in frontotemporal dementia: Heterogeneity and relationships with underlying neuropathology.. Neurotherapeutics 2021; 18 (02) 728-752 https://doi.org/10.1007/s13311-021-01101-x
- 39 Vijverberg EG, Wattjes MP, Dols A, Krudop WA, Moller C, Peters A. 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 2016; 53 (04) 1287-1297 https://doi.org/10.3233/jad-160285
- 40 Vijverberg EGB, Schouws S, Meesters PD, Verwijk E, Comijs H, Koene T. et al. Cognitive deficits in patients with neuropsychiatric symptoms: A comparative study between behavioral variant frontotemporal dementia and primary psychiatric disorders. J Clin Psychiatry 2017; 78 (08) e940-e6 https://doi.org/10.4088/jcp.16m11019
- 41 Grossman M, Farmer J, Leight S, Work M, Moore P, Van Deerlin V. et al. Cerebrospinal fluid profile in frontotemporal dementia and Alzheimer's disease. Ann Neurol 2005; 57 (05) 721-729 https://doi.org/10.1002/ana.20477
- 42 Bian H, Van Swieten JC, Leight S, Massimo L, Wood E, Forman M. et al. CSF biomarkers in frontotemporal lobar degeneration with known pathology. Neurology 2008; 70 (19) 02 1827-1835 https://doi.org/10.1212/01.wnl.0000311445.21321.fc
- 43 Greaves CV, Rohrer JD.. An update on genetic frontotemporal dementia. J Neurol 2019; 266 (08) 2075-2086 https://doi.org/10.1007/s00415-019-09363-4
- 44 Takada LT, Bahia VS, Guimarães HC, Costa TV, Vale TC, Rodriguez RD. et al. GRN and MAPT mutations in 2 frontotemporal dementia research centers in Brazil. Alzheimer Dis Assoc Disord 2016; 30 (04) 310-317 https://doi.org/10.1097/wad.2022s1402022s1400153
- 45 Gambogi LB, Guimaraes HC, de Souza LC, Caramelli P.. Treatment of the behavioral variant of frontotemporal dementia: A narrative review. Dement Neuropsychol 2021; 15 (03) 331-338
- 46 Galimberti D, Prunas C, Paoli RA, Dell'Osso B, Fenoglio C, Villa C. et al. Progranulin gene variability influences the risk for bipolar I disorder, but not bipolar II disorder. Bipolar Disord 2014; 16 (07) 769-772 https://doi.org/10.1111/bdi.12180
- 47 Cerami C, Marcone A, Galimberti D, Villa C, Scarpini E, Cappa SF.. From genotype to phenotype: Two cases of genetic frontotemporal lobar degeneration with premorbid bipolar disorder.. J Alzheimers Dis 2011; 27 (04) 791-797 https://doi.org/10.3233/jad-2011-110788
- 48 Devenney E, Hornberger M, Irish M, Mioshi E, Burrell J, Tan R. et al. Frontotemporal dementia associated with the C9ORF72 mutation: a unique clinical profile. JAMA Neurol 2014; 71 (03) 331-339 https://doi.org/10.1001/jamaneurol.2013.6002
- 49 Ducharme S, Dols A, Laforce R, Devenney E, Kumfor F, van den Stock J. et al. Recommendations to distinguish behavioural variant frontotemporal dementia from psychiatric disorders. Brain 2020; 143 (06) 1632-1650 https://doi.org/10.1093/brain/awaa018