Evaluation of patients with behavioral and cognitive complaints: Misdiagnosis in frontotemporal dementia and Alzheimer's disease

Beber, Bárbara Costa; Chaves, Márcia L.F.

doi:10.1590/S1980-57642013DN70100010

ABSTRACT

Background:

Frontotemporal dementia (FTD) is a heterogeneous clinicopathological syndrome whose early diagnosis is critical for developing management strategies.

Objective:

To analyze the variables associated with misdiagnosis in a group of patients with FTD, Alzheimer's disease (AD), and without neurodegenerative disorders (WND), all of whom were evaluated for behavioral and cognitive complaints.

Methods:

A case-control study with FTD (n=10), probable AD (n=10) and WND (n=10) patients was carried out. The studied variables were disease duration, reason for referral, former diagnosis, behavioral and cognitive symptoms at evaluation, MMSE at the specialist evaluation, and follow-up outcome. The data were analyzed by ANOVA with Bonferroni post-hoc and by Pearson's Chi-Square tests.

Results:

FTD patients and WND patients showed longer disease duration than AD patients; the main reasons for referral in the FTD group were behavioral, memory and memory plus language problems while all AD and 90% of the WND group were referred for memory. The FTD group had the highest rate of misdiagnosis and worst outcomes after the 12-month follow-up. The majority of AD and WND patients had memory symptoms, while FTD patients presented language (30%), memory and/or language (40%) problems on the evaluation.

Conclusion:

Difficulty in recognizing the main features of FTD and psychiatric disorders with memory impairment was observed. Clinicians tended to generalize memory complaints toward a single diagnosis, identifying almost all these patients as AD or leaving them undiagnosed.

Key words:
frontotemporal dementia; Alzheimer's disease; diagnosis

RESUMO

Introdução:

Demência frontotemporal (DFT) é uma síndrome clinicopatológica heterogênea e seu diagnóstico precoce é essencial para o desenvolvimento de estratégias de manejo.

Objetivo:

Analisar as variáveis associadas ao erro dignóstico em pacientes com DFT, doença de Alzheimer (DA) e sem transtornos neurodegenerativos (STN), avaliados por queixas cognitivas e comportamentais.

Métodos:

Estudo de caso-controle foi realizado com pacientes com DFT (n=10), provável DA (n=10), e STN (n=10). As variáveis estudadas foram duração da doença, motivo do encaminhamento, diagnóstico prévio, sintomas cognitivos e comportamentais na avaliação especializada, MEEM na avaliação, e desfecho. As análises foram feitas por ANOVA com Bonferroni post-hoc e Qui-Quadrado de Pearson.

Resultados:

Pacientes com DFT e STN mostraram maior tempo de duração da doença; os principais motivos de encaminhamento no grupo DFT foram problemas comportamentais, memória e memória mais linguagem, enquanto em todos pacientes com DA e 90% do grupo STN foi memória. O grupo DFT teve maiores taxas de erro diagnóstico e piores desfechos no seguimento de 12 meses. A maioria dos pacientes com DA e STN teve sintomas de memória, enquanto pacientes com DFT apresentaram sintomas de linguagem (30%), memória e/ou linguagem (40%) na avaliação.

Conclusão:

Dificuldade em reconhecer as principais características da DFT e de transtornos psiquiátricos com prejuízo de memória foi observada. Os clínicos tenderam a generalizar queixas de memória em direção a um único diagnóstico, identificando quase todos estes pacientes como tendo DA ou deixando-os sem diagnóstico.

Palavras-chave:
Demência Frontotemporal; doença de Alzheimer; diagnóstico

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REFERENCES

Mendez MF, Perryman KM. Neuropsychiatric Features of Frontotemporal Dementia: Evaluation of Consensus Criteria and Review. J Neuropsychiatry Clin Neurosci 2002;14:424-429.
Ratnavalli E, Brayne C, Dawson K, et al. The prevalence of frontotemporal dementia. Neurology 2002;58:1615-1621.
Rabinovici GD, Miller BL. Frontotemporal Lobar Degeneration: Epidemiology, Pathophysiology, Diagnosis and Management. CNS Drugs 2010; 24:375-398.
Fujihara S, Brucki S, Rocha MSG, Carvalho AA, Piccolo AC. Prevalence of presenile dementia in a tertiary outpatient clinic. Arq Neuropsiquiatr 2004;62:592-595.
Lund and Manchester Groups. Clinical and neuropathological criteria for frontotemporal dementia. J Neurol Neurosurg Psychiatry 1994;57:416-418.
Neary D, Snowden JS, Gustafson L, et al. Frontotemporal lobar degeneration: A consensus on clinical diagnostic criteria. Neurology1998; 51:1546-1554.
Gorno-Tempini ML, Hillis AE, Weintraub S, et al. Classification of primary progressive aphasia and its variants. Neurology2011;76:1006-1014.
Rascovsky K, Hodges JR, Knopman D, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain 2011;134:2456-2477.
Lindau M, Almkvist O, Kushi J, et al. First Symptoms - Frontotemporal Dementia versus Alzheimer's Disease. Dement Geriatr Cogn Disord 2000;11:286-293.
McKhann GM, Albert MS, Grossman M, Miller B, Dickson D, Trojanowski JQ. Clinical and Pathological Diagnosis of Frontotemporal Dementia: Report of the Work Group on Frontotemporal Dementia and Pick's Disease. Arch Neurol 2001;58:1803-1809.
Gregory CA, Hodges JR. Clinical features of frontal lobe dementia in comparison to Alzheimer's disease. J Neural Transm Suppl 1996;47: 103-123.
Passant U, Elfgren C, Englund E, Gustafson L. Psychiatric Symptoms and Their Psychosocial Consequences in Frontotemporal Dementia. Alz Dis Assoc Disord 2005;19:S15-S18.
Mendez MF, Shapira JS, McMurtray A, Licht, Miller BL. Accuracy of the clinical evaluation for frontotemporal dementia. Arch Neurol2007; 64:830-835.
McKhann G, Drachman D, Folstein M, et al. Clinical diagnosis of Alzheimer's disease: report of the NINCDS-ADRDA work group under the auspices of department of Health and Human Services Task Force on Alzheimer's disease. Neurology1984;34:939-944.
Woolley JD, Wilson MR, Hung E, et al. Frontotemporal dementia and mania. Am J Psychiatry 2007;164:1811-1816.
Varma AR, Snowden JS, Lloyd JJ, Talbot PR, Mann DMA, Neary D. Evaluation of the NINCDS-ADRDA criteria in the differentiation of Alzheimer's disease and frontotemporal dementia. J Neurol Neurosurg Psychiatry1999;66:184-188.
Bahia VS. Underdiagnosis of frontotemporal lobar degeneration in Brazil. Dement Neuropsychol 2007;1:361-365.
David HT, Robert CG, Kelly SB, Rebecca AS, Lindsay AF. Predictors of subjective memory complaint in cognitively normal relatives of patients with Alzheimer's disease. J Neuropsychiatry and Clinical Neurosciences 2006;18:384-388
Balash Y, Mordechovich M, Shabtai H, Giladi N, Gurevich T, Korczyn AD. Subjective memory complaints in elders: depression, anxiety, or cognitive decline? Acta Neurol Scand 2012; [Epub ahead of print]
Semkovska M, Noone M, Carton M, McLoughlin DM. Measuring consistency of autobiographical memory recall in depression. Psychiatry Res 2012;197:41-48.
Bonner MF, Ash S, Grossman S. The New Classification of Primary Progressive Aphasia into Semantic, Logopenic, or Nonfluent/Agrammatic Variants. Curr Neurol Neurosci Rep 2010 ;10:484-490.
Piguet O, Hornberger M, Mioshi E, Hodges JR. Behavioural-variant frontotemporal dementia: diagnosis, clinical staging, and management. Lancet Neurol 2011;10:162-172.
Caramelli P, Bottino CMC. Treating the behavioral and psychological symptoms of dementia (BPSD). J Bras Psiquiatr 2007;56: 83-87.
Yaffe K, Fox P, Newcomer R, et al. Patient and caregiver characteristics and nursing home placement in patients with dementia. JAMA 2002; 287:2090-2097.
Gorzoni ML, Pires SL. Clinical aspectcs of the senile dementia in long-term care facilities. Rev Psiq Clin 2006;33:18-23.
Andreasen N, Blennow K, Sjödin C, Winblad B, Svärdsudd K. Prevalence and incidence of clinically diagnosed memory impairments in a geographically defined general population in Sweden. The Piteå Dementia Project. Neuroepidemiology 1999;18:144-155.

Publication Dates

Publication in this collection
Jan-Mar 2013

History

Received
05 Nov 2012
Accepted
05 Jan 2013

This is an open-access article distributed under the terms of the Creative Commons Attribution License

[1] Mendez MF, Perryman KM. Neuropsychiatric Features of Frontotemporal Dementia: Evaluation of Consensus Criteria and Review. J Neuropsychiatry Clin Neurosci 2002;14:424-429.

[2] Ratnavalli E, Brayne C, Dawson K, et al. The prevalence of frontotemporal dementia. Neurology 2002;58:1615-1621.

[3] Rabinovici GD, Miller BL. Frontotemporal Lobar Degeneration: Epidemiology, Pathophysiology, Diagnosis and Management. CNS Drugs 2010; 24:375-398.

[4] Fujihara S, Brucki S, Rocha MSG, Carvalho AA, Piccolo AC. Prevalence of presenile dementia in a tertiary outpatient clinic. Arq Neuropsiquiatr 2004;62:592-595.

[5] Lund and Manchester Groups. Clinical and neuropathological criteria for frontotemporal dementia. J Neurol Neurosurg Psychiatry 1994;57:416-418.

[6] Neary D, Snowden JS, Gustafson L, et al. Frontotemporal lobar degeneration: A consensus on clinical diagnostic criteria. Neurology1998; 51:1546-1554.

[7] Gorno-Tempini ML, Hillis AE, Weintraub S, et al. Classification of primary progressive aphasia and its variants. Neurology2011;76:1006-1014.

[8] Rascovsky K, Hodges JR, Knopman D, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain 2011;134:2456-2477.

[9] Lindau M, Almkvist O, Kushi J, et al. First Symptoms - Frontotemporal Dementia versus Alzheimer's Disease. Dement Geriatr Cogn Disord 2000;11:286-293.

[10] McKhann GM, Albert MS, Grossman M, Miller B, Dickson D, Trojanowski JQ. Clinical and Pathological Diagnosis of Frontotemporal Dementia: Report of the Work Group on Frontotemporal Dementia and Pick's Disease. Arch Neurol 2001;58:1803-1809.

[11] Gregory CA, Hodges JR. Clinical features of frontal lobe dementia in comparison to Alzheimer's disease. J Neural Transm Suppl 1996;47: 103-123.

[12] Passant U, Elfgren C, Englund E, Gustafson L. Psychiatric Symptoms and Their Psychosocial Consequences in Frontotemporal Dementia. Alz Dis Assoc Disord 2005;19:S15-S18.

[13] Mendez MF, Shapira JS, McMurtray A, Licht, Miller BL. Accuracy of the clinical evaluation for frontotemporal dementia. Arch Neurol2007; 64:830-835.

[14] McKhann G, Drachman D, Folstein M, et al. Clinical diagnosis of Alzheimer's disease: report of the NINCDS-ADRDA work group under the auspices of department of Health and Human Services Task Force on Alzheimer's disease. Neurology1984;34:939-944.

[15] Woolley JD, Wilson MR, Hung E, et al. Frontotemporal dementia and mania. Am J Psychiatry 2007;164:1811-1816.

[16] Varma AR, Snowden JS, Lloyd JJ, Talbot PR, Mann DMA, Neary D. Evaluation of the NINCDS-ADRDA criteria in the differentiation of Alzheimer's disease and frontotemporal dementia. J Neurol Neurosurg Psychiatry1999;66:184-188.

[17] Bahia VS. Underdiagnosis of frontotemporal lobar degeneration in Brazil. Dement Neuropsychol 2007;1:361-365.

[18] David HT, Robert CG, Kelly SB, Rebecca AS, Lindsay AF. Predictors of subjective memory complaint in cognitively normal relatives of patients with Alzheimer's disease. J Neuropsychiatry and Clinical Neurosciences 2006;18:384-388

[19] Balash Y, Mordechovich M, Shabtai H, Giladi N, Gurevich T, Korczyn AD. Subjective memory complaints in elders: depression, anxiety, or cognitive decline? Acta Neurol Scand 2012; [Epub ahead of print]

[20] Semkovska M, Noone M, Carton M, McLoughlin DM. Measuring consistency of autobiographical memory recall in depression. Psychiatry Res 2012;197:41-48.

[21] Bonner MF, Ash S, Grossman S. The New Classification of Primary Progressive Aphasia into Semantic, Logopenic, or Nonfluent/Agrammatic Variants. Curr Neurol Neurosci Rep 2010 ;10:484-490.

[22] Piguet O, Hornberger M, Mioshi E, Hodges JR. Behavioural-variant frontotemporal dementia: diagnosis, clinical staging, and management. Lancet Neurol 2011;10:162-172.

[23] Caramelli P, Bottino CMC. Treating the behavioral and psychological symptoms of dementia (BPSD). J Bras Psiquiatr 2007;56: 83-87.

[24] Yaffe K, Fox P, Newcomer R, et al. Patient and caregiver characteristics and nursing home placement in patients with dementia. JAMA 2002; 287:2090-2097.

[25] Gorzoni ML, Pires SL. Clinical aspectcs of the senile dementia in long-term care facilities. Rev Psiq Clin 2006;33:18-23.

[26] Andreasen N, Blennow K, Sjödin C, Winblad B, Svärdsudd K. Prevalence and incidence of clinically diagnosed memory impairments in a geographically defined general population in Sweden. The Piteå Dementia Project. Neuroepidemiology 1999;18:144-155.

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