Very late-onset behavioral variant frontotemporal dementia

Guimarães, Henrique Cerqueira; Espindola, Tatiana de Carvalho

doi:10.1590/S1980-57642013DN70100020

ABSTRACT

Current concepts regarding frontotemporal lobar degeneration (FTLD) have evolved rapidly in recent years. Genetically determined FTLD cohorts have broadened our knowledge pertaining to its clinical presentation, neuroimaging findings and demographics. In this study we present a case report of a patient diagnosed with behavioral variant frontotemporal dementia diagnosis in her nineties during hospital admission for a ground-level fall. We believe this case reinforces the pervasive nature of this clinical entity, and may contribute to an increased awareness of this diagnostic possibility in late-onset dementia.

Key words:
frontotemporal dementia; very late-onset; dementia; diagnosis

RESUMO

Os conceitos atuais à respeito da degeneração lobar frontotemporal (DLFT) evoluíram rapidamente nos últimos anos. Coortes de pacientes com DLFT geneticamente determinada ampliaram nossos conhecimentos a cerca dos aspectos clínicos, de neuroimagem e demográficos relacionados a esta doença. Aqui nós reportamos o caso de uma paciente que recebeu o diagnóstico de demência frontotemporal em sua variante comportamental aos noventa anos de idade, por ocasião de uma internação hospitalar devido à queda da própria altura. Este caso reforça a ubiquidade desta entidade clínica, e pode contribuir para a conscientização quanto a esta possibilidade diagnóstica em demências de início tardio.

Palavras-chave:
demência frontotemporal; início muito tardio; demência; diagnóstico

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REFERENCES

Keith JA, Hodges JR, Snowden JS, et al. Neuropathological background of phenotypical variability in frontotemporal dementia. Acta Neuropathol 2011;122:137-153.
Van Lagenhove T, van der Zee J, Gijselinck I, et al. Distinct clinical characteristics C9orf72 expansion carriers compared with GRN, MATP, and no mutation carriers in a Flanders-Belgian FTLD cohort. JAMA Neurol 2013[epub ahead of print] doi:10.1001/2013.
Lund-Manchester clinical and neuropathological criteria for frontotemporal dementia. The Lund and Manchester Groups. J Neurol Neurosurg Psychiatry 1994;57:416-418.
Johnson JK, Diehl J, Mendez MF, et al. Frontotemporal lobar degeneration. Arch Neurol 2005;62:925-930.
Whitwell JL, Weigand SD, Boeve B, et al. Neuroimaging signatures of frontotemporal dementia genetics: C9ORF72, tau, progranulin, sporadics. Brain 2012;135:794-806.
Hornberger M, Piguet O. Episodic memory in frontotemporal dementia: a critical review. Brain2012;135:678-692.
Hodges J. Familial frontotemporal dementia and amyotrophic lateral sclerosis associated with C9orf72 hexanucelotide repeat. Brain 2012; 135:652-655.
Bruni A, Momeni P, Bernardi L, et al. heterogeneity within a large kindred with frontotemporal dementia: a novel progranulin mutation. Neurology 2007;69:140-147.
Fried LP, Tangen CM, Waltson J, et al. Frailty in older adults: evidence for a phenotype. J Gerontol A Biol Sci Med Sci 2001;56:146-156.
Katz S, Downs TD, Cash HR, Grotz RC. Progress in the development of the index of ADL. Gerontologist 1970;10:20-30.
Pfeffer RI, Kurosaki TT, Harrah CH, et al. Measurement of functional activities in older adults in the community. J Gerontol 1982;37:323-329.
Mioshi E, Hsieh S, Savage S, Hornberger M, Hodges JR. Clinical staging and disease progression in frontotemporal dementia. Neurology2010;74:1591-1597.
Gislason TB, Sjögren M, Larsson L, Skoog I. The prevalence of frontal variant frontemporal dementia and the frontal lobe syndrome in a population based sample of 85 year olds. J Neurol Neurosurg Psychiatr 2003;74:867-871.
Baborie A, Griffiths TD, Jaros E, et al. Pathological correlates of frontotemporal lobar degeneration in the elderly. Acta Neuropathol2011; 121:365-371.
Borroni B, Agosti C, Bellelli G, Padovani A. Is early-onset clinically different from late-onset frontotemporal dementia? Eur J Neurol 2008;15: 1412-1415.
Rosso SM, Kaak 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.
Gass J, Cannon A, Mackenzie IR, et al. Mutations in progranulin are a major cause of ubiquitin-positive frontotemporal lobar degeneration. Hum Mol Genet 2006;15:2988-23001.
Bahia VS. Underdiagnosis of frontotemporal lobar degeneration in Brazil. Dement Neuropsychol 2007;1:361-365.

Publication Dates

Publication in this collection
Jan-Mar 2013

History

Received
15 Nov 2012
Accepted
20 Jan 2013

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

[1] Keith JA, Hodges JR, Snowden JS, et al. Neuropathological background of phenotypical variability in frontotemporal dementia. Acta Neuropathol 2011;122:137-153.

[2] Van Lagenhove T, van der Zee J, Gijselinck I, et al. Distinct clinical characteristics C9orf72 expansion carriers compared with GRN, MATP, and no mutation carriers in a Flanders-Belgian FTLD cohort. JAMA Neurol 2013[epub ahead of print] doi:10.1001/2013.

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

[4] Johnson JK, Diehl J, Mendez MF, et al. Frontotemporal lobar degeneration. Arch Neurol 2005;62:925-930.

[5] Whitwell JL, Weigand SD, Boeve B, et al. Neuroimaging signatures of frontotemporal dementia genetics: C9ORF72, tau, progranulin, sporadics. Brain 2012;135:794-806.

[6] Hornberger M, Piguet O. Episodic memory in frontotemporal dementia: a critical review. Brain2012;135:678-692.

[7] Hodges J. Familial frontotemporal dementia and amyotrophic lateral sclerosis associated with C9orf72 hexanucelotide repeat. Brain 2012; 135:652-655.

[8] Bruni A, Momeni P, Bernardi L, et al. heterogeneity within a large kindred with frontotemporal dementia: a novel progranulin mutation. Neurology 2007;69:140-147.

[9] Fried LP, Tangen CM, Waltson J, et al. Frailty in older adults: evidence for a phenotype. J Gerontol A Biol Sci Med Sci 2001;56:146-156.

[10] Katz S, Downs TD, Cash HR, Grotz RC. Progress in the development of the index of ADL. Gerontologist 1970;10:20-30.

[11] Pfeffer RI, Kurosaki TT, Harrah CH, et al. Measurement of functional activities in older adults in the community. J Gerontol 1982;37:323-329.

[12] Mioshi E, Hsieh S, Savage S, Hornberger M, Hodges JR. Clinical staging and disease progression in frontotemporal dementia. Neurology2010;74:1591-1597.

[13] Gislason TB, Sjögren M, Larsson L, Skoog I. The prevalence of frontal variant frontemporal dementia and the frontal lobe syndrome in a population based sample of 85 year olds. J Neurol Neurosurg Psychiatr 2003;74:867-871.

[14] Baborie A, Griffiths TD, Jaros E, et al. Pathological correlates of frontotemporal lobar degeneration in the elderly. Acta Neuropathol2011; 121:365-371.

[15] Borroni B, Agosti C, Bellelli G, Padovani A. Is early-onset clinically different from late-onset frontotemporal dementia? Eur J Neurol 2008;15: 1412-1415.

[16] Rosso SM, Kaak 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.

[17] Gass J, Cannon A, Mackenzie IR, et al. Mutations in progranulin are a major cause of ubiquitin-positive frontotemporal lobar degeneration. Hum Mol Genet 2006;15:2988-23001.

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

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