Underdiagnosis of frontotemporal lobar degeneration in Brazil

Bahia, Valéria Santoro

doi:10.1590/S1980-57642008DN10400006

Abstract

Frontotemporal lobar degeneration (FTLD) is a progressive neurodegenerative disorder that involves the frontal and anterior temporal lobes. It is characterized by prominent behavioral and language disorders. There are three variants of FTLD: frontotemporal dementia (FTD), semantic dementia (SD) and progressive non-fluent aphasia (PNFA). FTLD may be mistaken for AD (Alzheimer's disease) or psychiatric disorders especially in the early stages. Objectives: To describe the demographic characteristics of patients with FTLD, assessed at the Behavioral and Cognitive Neurology Unit, and to show that FTLD is commonly clinically under-diagnosed. Methods: All patients diagnosed with FTLD (Consensus Criteria for FTLD), and who were seen at the Behavioral and Cognitive Neurology Unit of Hospital das Clínicas, in São Paulo, Brazil from January 2004 to August 2007 were included in the analyses. Results: Sixteen patients with FTLD (11 women) were included in this study. There were 12 patients with FTD, two with PNFA and two with SD. The mean duration of the illness until diagnosis of FTLD was 4.1±2.3 years, ranging from one to seven years. Nine patients had been initially seen by psychiatrists, five by neurologists, and two by general physicians. The first diagnosis was psychiatric disorder in six patients, AD in four, dementia in two, FTD in two, and stroke and hydrocephalus for one patient each. Conclusion: The diagnosis of FTLD can be difficult and many patients may be misdiagnosed in Brazil, especially in the initial stages. Educational programs on FTLD for the medical community are warranted.

Key words:
frontotemporal lobar degeneration; frontotemporal dementia; Alzheimer's disease; semantic dementia; primary progressive aphasia

Resumo

Degeneração Lobar Fronto-temporal (DLFT) é uma doença neurodegenerativa progressiva que acomete lobos frontais e temporais. Caracteriza-se pela predominância de distúrbios comportamentais e de linguagem. Há três variantes da DLFT: demência fronto-temporal (DFT), demência semântica (DS) e afasia progressiva não fluente (APNF). Pacientes com DLFT podem ser diagnosticados erroneamente, principalmente nas fases iniciais. Objetivos: Descrever as características demográficas dos pacientes avaliados no Grupo de Neurologia Cognitiva e do Comportamento e mostrar que os casos de DLFT são freqüentemente diagnosticados erroneamente em fase inicial. Métodos: Avaliamos todos os indivíduos com o diagnóstico de DLFT, (Critérios de Consenso de DLFT) atendidos no Grupo de Neurologia Cognitiva e do Comportamento do Hospital das Clínicas, São Paulo, Brasil, entre Janeiro de 2004 e Agosto de 2007. Resultados: Foram incluídos 16 pacientes com DLFT (11 mulheres). Doze pacientes tiveram o diagnóstico de DFT, dois de DS e dois APNF. A média de duração da doença até o diagnóstico de DLFT foi de 4,1±2,3 anos (variando de um a sete anos). Nove pacientes foram avaliados, no início da doença, por psiquiatras, cinco por neurologistas e dois por clínicos gerais. O primeiro diagnóstico foi de distúrbio psiquiátrico em seis pacientes, DA em quatro pacientes, demência em dois, DFT em dois, acidente vascular cerebral em um e hidrocefalia em um paciente. Conclusão: O diagnóstico de DLFT pode ser difícil e muitos pacientes, principalmente em fases iniciais, podem estar sendo diagnosticados erroneamente no Brasil. Há a necessidade de maior informação sobre a doença na comunidade médica.

Palavras-chave:
degeneração lobar fronto-temporal; demência fronto-temporal; doença de Alzheimer; demência semântica; afasia progressiva primária

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References

¹
Neary D, Snowden JS, Gustafson L, et al. Frontotemporal lobar degeneration. A consensus on clinical diagnostic criteria. Neurology 1998;51:1546-1554.
²
Neary D, Snowden J, Mann D. Frontotemporal dementia. Lancet Neurol 2005;4:771-780.
³
Ratnavalli E, Brayne C, Dawson K, Hodges JR. The prevalence of frontotemporal dementia. Neurology 2002;58:1615-1621.
⁴
Rosso SM, Kaat LD, Baks T, et al. Frontotemporal dementia in The Netherlands: patient characteristics and prevalence estimates from a population-based study. Brain 2003;126:2016-2022.
⁵
Johnson JK, Dichl J, Mendez MF, et al. Frontotemporal lobar degeneration. Demographic characteristics of 353 patients. Arch Neurol 2005;62:925-930.
⁶
Harvey RJ, Skelton-Robinson M, Rossor MN. The prevalence and causes of dementia in people under the age of 65 years. J Neurol Neurosurg Psychiatry 2003;74:1206-1209.
⁷
Herrera E Jr, Caramelli, Silveira AS, Nitrini R. Epidemiologic survey of dementia in a community-dwelling Brazilian population. Alzheimer Dis Assoc Disord. 2002;16:103-108.
⁸
Snowden JS, Neary D, Mann DMA. Frontotemporal dementia. Br J Psychiatry 2002;180:140-143.
⁹
Snowden JS, Bathgate D, Varma A, Blackshaw A, Gibbons ZC, Neary D. Distinct bahavioral profiles in frontotemporal dementia and semantic dementia. J Neurol Neurosurg Psychiatry 2001;70:323-332.
¹⁰
Gorno-Tempini ML, Dronkers NF, Rankin KP et al. Cognition and anatomy in three variants of primary progressive aphasia. Ann Neurol 2004;55:335-46.
¹¹
Rosen HJ, Allison SC, Ogar JM, et al. Behavioral features in semantic dementia vs other forms of progressive aphasias. Neurology 2006;67:1752-1756.
¹²
Rosen HJ, Hartikainen KM, Jagust W, et al. Utility of clinical criteria in differentiating frontotemporal lobar degeneration (FTLD) from AD. Neurology 2002;58:1608-1615.
¹³
Liscic RM, Storandt M, Cairns NJ, Morris JC. Clinical and Psychometric distinction of frontotemporal and Alzheimer dementias. Arch Neurol 2007;64:535-540.
¹⁴
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 Psychiatry 1999;66:184-188.
¹⁵
Mendez MF, Shapira JS, McMurtray A, Licht E, Miller B. Accuracy of the clinical evaluation for frontotemporal dementia. Arch Neurol 2007;64:830-835.
¹⁶
Nitrini R, Lefèvre BH, Mathias SC, et al. Testes neuropsicológicos de aplicação simples para o diagnóstico de demência. Arq Neuropsiquiatr 1994;52:457-465.
¹⁷
Mattis S. Mental Status Examination for Organic Mental Syndrome in the Elderly Patient. In: Bellak L, Karasu TB, editors. Geriatric Psychiatry. A Handbook for Psychiatrists and Primary Care Physicians. New York: Grune & Stratton; 1976:77-121.
¹⁸
Dubois B, Slachevsky A, Litvan I, Pillon B. The FAB: A Frontal Assessment Battery at bedside. Neurology 2000;55:1621-1626.
¹⁹
Pfeffer RI, Kusosaki TT, Harrah Jr CH, Chance JM, Filos S. Measurement of functional activities in older adults in the community. J Gerontol 1982;37:323-329.
²⁰
Caine D, Patterson K, Hodges JR, Heard R, Halliday G. Severe anterograde amnesia with extensive hippocampal degeneration in a case of rapidly progressive frontotemporal dementia. Neurocase 2001;7:57-64.
²¹
Galton CJ, Patterson K, Graham K, et al. Differing patterns of temporal atrophy in Alzheimer's disease and semantic dementia. Neurology 2001;57:216-225.
²²
Graham A, Davies R, Xuereb J, et al. Pathologically proven frontotemporal dementia presenting with severe amnesia. Brain 2005;128:597-605.
²³
Bozeat S, Gregory CA, Lambon Ralph MA et al. Which neuropsychiatry and behavioural features distinguish frontal and temporal variants of frontotemporal dementia from Alzheimer's disease? J Neurol Neurosurg Psychiatry 2000;69:178-186.
²⁴
Nyatsanza S, Shetty T, Gregory C, Dawson K, Hodges JR. A study of stereotypic behaviours in Alzheimer's disease and frontal and temporal variant frontotemporal dementia. J Neurol Neurosurg Psychiatry 2003;74:1398-1402.
²⁵
Shinagawa S, Ikeda M, Fukuhara R, Tanabe H. Initial symptoms in frontotemporal dementia and semantic dementia compared with Alzheimer's disease. Dement Geriatr Cogn Disord 2006;21:74-80.
²⁶
Kertesz A, Davidson W, McCabe P, Munoz D. Behavioral quantitation is more sensitive than cognitive testing in frontotemporal dementia. Alzheimer Dis Assoc Disord 2003;17:223-229
²⁷
Hutchinson AD, Mathias JL. Neuropsychological deficits in frontotemporal dementia and Alzheimer's disease: a meta-analytic review. J Neurol Neurosurg Psychiatry. 2007;78:917-928.
²⁸
Diehl-Schmid J, Pohl C, Perneczky R, Förstl H, Kurz A. Behavioral disturbances in the course of frontotemporal dementia. Dement Geriatr Cogn Disord 2006;22:352-357.
²⁹
Saykin AJ, Gur RC, Gur RE, et al. Neuropsychological function in schizophrenia: selective impairment in memory and learning. Arch Geriatr Psychiatry 1991;48:618-624.
³⁰
Vries PJ, Honer WG, Kemp PM, McKenna PJ. Dementia as a complication of schizophrenia. J Neurol Neurosurg Psychiatry 2001:70:588-596.
³¹
Oliveira-Souza R, Marrocos RP, Moll J. The dementias of schizophrenia. Dement Neuropsychol 2007;2:124-30.
³²
Waddington JL, Youssef HA, Farrel MA, Toland J. Initial 'schizophrenia-like' psychosis in Pick's disease: case study with neuroimaging and neuropathology, and implications for frontotemporal dysfunction in schizophrenia. Schizophrenia Research 1995;18:79-82.
³³
Stone J, Griffiths TD, Rastogi S, Perry RH, Cleland PG. Non-Picks frontotemporal dementia imitating schizophrenia in a 22-year-old man. J Neurol 2003;250:369-370.
³⁴
Kitabayashi Y, Otakara C, Hirosawa R, Narumoto J, Fukui K. Frontotemporal dementia complicated with schizophrenia. Psychiatry and Clinical Neurosciences 2005;59:749-750.

Publication Dates

Publication in this collection
Oct-Dec 2007

History

Received
25 Oct 2007
Reviewed
05 Nov 2007
Accepted
18 Nov 2007

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

[1] ¹
Neary D, Snowden JS, Gustafson L, et al. Frontotemporal lobar degeneration. A consensus on clinical diagnostic criteria. Neurology 1998;51:1546-1554.

[2] ²
Neary D, Snowden J, Mann D. Frontotemporal dementia. Lancet Neurol 2005;4:771-780.

[3] ³
Ratnavalli E, Brayne C, Dawson K, Hodges JR. The prevalence of frontotemporal dementia. Neurology 2002;58:1615-1621.

[4] ⁴
Rosso SM, Kaat LD, Baks T, et al. Frontotemporal dementia in The Netherlands: patient characteristics and prevalence estimates from a population-based study. Brain 2003;126:2016-2022.

[5] ⁵
Johnson JK, Dichl J, Mendez MF, et al. Frontotemporal lobar degeneration. Demographic characteristics of 353 patients. Arch Neurol 2005;62:925-930.

[6] ⁶
Harvey RJ, Skelton-Robinson M, Rossor MN. The prevalence and causes of dementia in people under the age of 65 years. J Neurol Neurosurg Psychiatry 2003;74:1206-1209.

[7] ⁷
Herrera E Jr, Caramelli, Silveira AS, Nitrini R. Epidemiologic survey of dementia in a community-dwelling Brazilian population. Alzheimer Dis Assoc Disord. 2002;16:103-108.

[8] ⁸
Snowden JS, Neary D, Mann DMA. Frontotemporal dementia. Br J Psychiatry 2002;180:140-143.

[9] ⁹
Snowden JS, Bathgate D, Varma A, Blackshaw A, Gibbons ZC, Neary D. Distinct bahavioral profiles in frontotemporal dementia and semantic dementia. J Neurol Neurosurg Psychiatry 2001;70:323-332.

[10] ¹⁰
Gorno-Tempini ML, Dronkers NF, Rankin KP et al. Cognition and anatomy in three variants of primary progressive aphasia. Ann Neurol 2004;55:335-46.

[11] ¹¹
Rosen HJ, Allison SC, Ogar JM, et al. Behavioral features in semantic dementia vs other forms of progressive aphasias. Neurology 2006;67:1752-1756.

[12] ¹²
Rosen HJ, Hartikainen KM, Jagust W, et al. Utility of clinical criteria in differentiating frontotemporal lobar degeneration (FTLD) from AD. Neurology 2002;58:1608-1615.

[13] ¹³
Liscic RM, Storandt M, Cairns NJ, Morris JC. Clinical and Psychometric distinction of frontotemporal and Alzheimer dementias. Arch Neurol 2007;64:535-540.

[14] ¹⁴
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 Psychiatry 1999;66:184-188.

[15] ¹⁵
Mendez MF, Shapira JS, McMurtray A, Licht E, Miller B. Accuracy of the clinical evaluation for frontotemporal dementia. Arch Neurol 2007;64:830-835.

[16] ¹⁶
Nitrini R, Lefèvre BH, Mathias SC, et al. Testes neuropsicológicos de aplicação simples para o diagnóstico de demência. Arq Neuropsiquiatr 1994;52:457-465.

[17] ¹⁷
Mattis S. Mental Status Examination for Organic Mental Syndrome in the Elderly Patient. In: Bellak L, Karasu TB, editors. Geriatric Psychiatry. A Handbook for Psychiatrists and Primary Care Physicians. New York: Grune & Stratton; 1976:77-121.

[18] ¹⁸
Dubois B, Slachevsky A, Litvan I, Pillon B. The FAB: A Frontal Assessment Battery at bedside. Neurology 2000;55:1621-1626.

[19] ¹⁹
Pfeffer RI, Kusosaki TT, Harrah Jr CH, Chance JM, Filos S. Measurement of functional activities in older adults in the community. J Gerontol 1982;37:323-329.

[20] ²⁰
Caine D, Patterson K, Hodges JR, Heard R, Halliday G. Severe anterograde amnesia with extensive hippocampal degeneration in a case of rapidly progressive frontotemporal dementia. Neurocase 2001;7:57-64.

[21] ²¹
Galton CJ, Patterson K, Graham K, et al. Differing patterns of temporal atrophy in Alzheimer's disease and semantic dementia. Neurology 2001;57:216-225.

[22] ²²
Graham A, Davies R, Xuereb J, et al. Pathologically proven frontotemporal dementia presenting with severe amnesia. Brain 2005;128:597-605.

[23] ²³
Bozeat S, Gregory CA, Lambon Ralph MA et al. Which neuropsychiatry and behavioural features distinguish frontal and temporal variants of frontotemporal dementia from Alzheimer's disease? J Neurol Neurosurg Psychiatry 2000;69:178-186.

[24] ²⁴
Nyatsanza S, Shetty T, Gregory C, Dawson K, Hodges JR. A study of stereotypic behaviours in Alzheimer's disease and frontal and temporal variant frontotemporal dementia. J Neurol Neurosurg Psychiatry 2003;74:1398-1402.

[25] ²⁵
Shinagawa S, Ikeda M, Fukuhara R, Tanabe H. Initial symptoms in frontotemporal dementia and semantic dementia compared with Alzheimer's disease. Dement Geriatr Cogn Disord 2006;21:74-80.

[26] ²⁶
Kertesz A, Davidson W, McCabe P, Munoz D. Behavioral quantitation is more sensitive than cognitive testing in frontotemporal dementia. Alzheimer Dis Assoc Disord 2003;17:223-229

[27] ²⁷
Hutchinson AD, Mathias JL. Neuropsychological deficits in frontotemporal dementia and Alzheimer's disease: a meta-analytic review. J Neurol Neurosurg Psychiatry. 2007;78:917-928.

[28] ²⁸
Diehl-Schmid J, Pohl C, Perneczky R, Förstl H, Kurz A. Behavioral disturbances in the course of frontotemporal dementia. Dement Geriatr Cogn Disord 2006;22:352-357.

[29] ²⁹
Saykin AJ, Gur RC, Gur RE, et al. Neuropsychological function in schizophrenia: selective impairment in memory and learning. Arch Geriatr Psychiatry 1991;48:618-624.

[30] ³⁰
Vries PJ, Honer WG, Kemp PM, McKenna PJ. Dementia as a complication of schizophrenia. J Neurol Neurosurg Psychiatry 2001:70:588-596.

[31] ³¹
Oliveira-Souza R, Marrocos RP, Moll J. The dementias of schizophrenia. Dement Neuropsychol 2007;2:124-30.

[32] ³²
Waddington JL, Youssef HA, Farrel MA, Toland J. Initial 'schizophrenia-like' psychosis in Pick's disease: case study with neuroimaging and neuropathology, and implications for frontotemporal dysfunction in schizophrenia. Schizophrenia Research 1995;18:79-82.

[33] ³³
Stone J, Griffiths TD, Rastogi S, Perry RH, Cleland PG. Non-Picks frontotemporal dementia imitating schizophrenia in a 22-year-old man. J Neurol 2003;250:369-370.

[34] ³⁴
Kitabayashi Y, Otakara C, Hirosawa R, Narumoto J, Fukui K. Frontotemporal dementia complicated with schizophrenia. Psychiatry and Clinical Neurosciences 2005;59:749-750.

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