Primary progressive aphasia: Classification of variants in 100 consecutive Brazilian cases

Senaha, Mirna Lie Hosogi; Caramelli, Paulo; Brucki, Sonia M.D.; Smid, Jerusa; Takada, Leonel T.; Porto, Claudia S.; César, Karolina G.; Matioli, Maria Niures P.; Soares, Roger T.; Mansur, Letícia L.; Nitrini, Ricardo

doi:10.1590/S1980-57642013DN70100017

ABSTRACT

Primary progressive aphasia (PPA) is a neurodegenerative clinical syndrome characterized primarily by progressive language impairment. Recently, consensus diagnostic criteria were published for the diagnosis and classification of variants of PPA. The currently recognized variants are nonfluent/agrammatic (PPA-G), logopenic (PPA-L) and semantic (PPA-S).

Objective:

To analyze the demographic data and the clinical classification of 100 PPA cases.

Methods:

Data from 100 PPA patients who were consecutively evaluated between 1999 and 2012 were analyzed. The patients underwent neurological, cognitive and language evaluation. The cases were classified according to the proposed variants, using predominantly the guidelines proposed in the consensus diagnostic criteria from 2011.

Results:

The sample consisted of 57 women and 43 men, aged at onset 67.2±8.1 years (range of between 53 and 83 years). Thirty-five patients presented PPA-S, 29 PPA-G and 16 PPA-L. It was not possible to classify 20% of the cases into any one of the proposed variants.

Conclusion:

It was possible to classify 80% of the sample into one of the three PPA variants proposed. Perhaps the consensus classification requires some adjustments to accommodate cases that do not fit into any of the variants and to avoid overlap where cases fit more than one variant. Nonetheless, the established current guidelines are a useful tool to address the classification and diagnosis of PPA and are also of great value in standardizing terminologies to improve consistency across studies from different research centers.

Key words:
primary progressive aphasia; clinical consensus; variants; agrammatic; logopenic; semantic; semantic dementia

RESUMO

A afasia progressiva primária (APP) é uma síndrome clínica neurodegenerativa caracterizada pelo comprometimento predominante e progressivo da linguagem. Recentemente, foi publicado um consenso clínico para o diagnóstico e classificação das variantes da APP. As variantes reconhecidas atualmente são: não-fluente/agramática (APP-G), logopênica (APP-L) e semântica (APP-S).

Objetivo:

Analisar os dados demográficos e classificar as variantes de uma amostra de 100 casos de APP.

Métodos:

Foram analisados os achados de 100 pacientes de APP que foram encaminhados consecutivamente para avaliação fonoaudiológica entre 1999 e 2012. Os pacientes foram submetidos à avaliação neurológica, cognitiva e de linguagem. A partir, principalmente, dos critérios elaborados pelo consenso clinico de APP, os casos foram classificados em uma das variantes.

Resultados:

Cem casos, 43 homens e 57 mulheres, foram avaliados. A idade de início variou entre 53 e 83 anos (x=67.2 (±8.1). Foram identificados 35 casos de APP-S, 29 de APP-G e 16 de APP-L. Vinte casos não se enquadraram em nenhumas das três variantes.

Conclusão:

Foi possível classificar distúrbio de linguagem em 80% da amostra em uma das três variantes de APP. A recomendação atual estabelecida pelo consenso clínico é uma ferramenta útil para direcionar a classificação e diagnóstico da APP e também é de grande valor para uniformidade das terminologias entre os diferentes centros de pesquisa. Porém, alguns ajustes seriam interessantes para contemplar os casos que não se encaixam em nenhuma das variantes e para evitar a sobreposição de casos que poderiam se encaixar em mais de uma variante.

Palavras-chave:
afasia progressiva primária; consenso clínico; variantes; agramática; logopênica; semântica; demência semântica

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REFERENCES

Mesulam MM. Slowly progressive aphasia without generalized dementia. Ann Neurol 1982;11:592-598.
Harciarik M, Kertesz, A. Primary progressive aphasias and their contribution to the contemporary knowledge about the brain-language relationship. Neuropsychol Rev 2011;21:271-287.
Mesulam MM, Grossman M, Hillis A; Kertesz A, Weintraub S. The core and halo of primary progressive aphasia and semantic dementia. Ann Neurol2003;54(suppl 5):S11-S14.
Knibb JA, Hodges JH. Semantic dementia and primary progressive aphasia: a problem of categorization. Alzheimer Dis Assoc Disord 2005; 19(suppl 1):S7-S14.
Adlam ALR, Patterson K, Rogers TT, et al. Semantic dementia and fluent primary progressive aphasia: two sides of the same coin? Brain 2006;129:3066-3080.
Rogaslki E, Mesulam M. An update on primary progressive aphasia. Curr Neurol Neurosci Rep 2007;7:388-392.
Gorno-Tempini ML, Hillis AE, Weintraub S, et al. Classification of primary progressive aphasia and its variants. Neurology 2011;76:1006- 1014.
Mesulam MM. Primary progressive aphasia. Ann Neurol2001;49:425-432.
Mesulam MM. Primary progressive aphasia - a language-based dementia. New Engl J Med 2003;349:1535-1542.
Gorno-Tempini ML, Dronkers NF, Rankin KP, et al. Cognition and Anatomy of three variants of primary progressive aphasia. Ann Neurol2004;55:335-346.
Desgranges B, Matuszewski V, Piolino P, et al. Anatomical and functional alterations in semantic dementia: a voxel-based MRI and PET study. Neurobiol Aging 2007;28:1904-1913.
Knibb JA, Xuereb JH, Patterson K, Hodges JR. Clinical and pathological characterization of progressive aphasia. Ann Neurol2006;59:156-165.
Hodges JR, Mitchell J, Dawson K, et al. Semantic dementia: demography, familial factors and survival in a consecutive series of 100 cases. Brain2010;133:300-306.
Grossman M. Primary progressive aphasia: clinicopathological correlations. Nat Rev Neurol 2010;6:88-97.
Leyton CE, Villemagne VL, Savage S, et al. Subtypes of progressive aphasia: application of the International Consensus Criteria and validation using b-amyloid imaging. Brain2011;134:3030-3043.
Nespoulous JL, Lecours AR, Lafond D, Parente MAMP. Protocole Montréal-Tolouse MT-86 d'examen linguistique de l'aphasie - version Beta. Laboratoire Théophile-Alajouanine, Montréal; 1986.
Goodglass H, Kaplan E. The Assessment of aphasia and related disorders. 2nd, Philadelphia, PA, USA: Lea & Febiger; 1993.
Kaplan E, Goodglass H, Weintraub S.The Boston Naming Test. Philadelphia: Lea & Febiger; 1983.
Parente MAMP, Hosogi ML, Delgado AP, Lecours, AR. Protocolo de Leitura para o projeto H.F.S.P. (Human Frontier Science Program); 1992.
Senaha MLH, Caramelli P, Porto CS, Nitrini R. Verbal and non-verbal semantic impairment: from fluent primary progressive aphasia to semantic dementia. Dement Neuropsychol 2007;2:203-211.
Senaha MLH, Caramelli P, Porto CS, Nitrini R. Semantic dementia Brazilian study of nineteen cases. Dement Neuropsychol2007;1:366-373.
Darley FL, Aronson AE, Brown JR. Motor speech disorders. Philadelphia: W.B. Saunders; 1975.
Agniel A, Joanette Y, Dojon B, Duchein C. Protocole d'évaluation des gnosies visuelles. Montréal-Toulouse. Isbergues: Ortho-édition; 1992.
Howard D, Patterson K. Pyramids and palm trees: a test of semantic access from pictures and words. Bury St Edmunds, Suffolk: Thames Valley Test Company; 1992.
Otsuki M, Soma Y, Sato M, Homma A, Tsuji S. Slowly Progressive Pure Word Deafness. Eur Neurol 1998;39:135-140.
Gorno-Tempini ML, Brambati SM, Ginex V, et al. The logopenic/phonological variant of primary progressive aphasia. Neurology2008;71:1227-1234.
Henry ML,. Gorno-Tempini ML The logopenic variant of primary progressive aphasia. Curr Opin Neurol 2010;23:633-637.
Rohrer JD, Rossor MN, Warren JD. Neologistic jargon aphasia and agraphia in primary progressive aphasia. J Neurol Sci 2009;277:155-159.
Folstein MF, Folstein SE, Mchugh PR. Mini-mental state. A practical method for grading the cognitive state of the patients for the clinician. J Psychiatry Res 1975;12:189-198.
Brucki SM, Nitrini R, Caramelli P, Bertolucci PH, Okamoto IH. Suggestions for utilization of the mini-mental state examination in Brazil. Arq Neuropsiquiatr 2003;61:777-781.
Mesulam M, Wieneke C, Rogalski E, Cobia D, Thompson C, Weintraub S.Quantitative template for subtyping primary progressive aphasia. Arch Neurol 2009;66:1545-1551.
Mesulam MM, Wieneke C , Thompson C, Rogalski E, Weintraub S.Quantitative classification of primary progressive aphasia at early and mild impairment stages. Brain2012;135:1537-1553.
Mesulam MM, Weintraub S.Primary progressive aphasia: sharpening the focus on a clinical syndrome. In: Boller, F et al. (eds.) Heterogeneuty of Alzheimer's disease. Springer-Verlag. Berlim Heiderlberg;1992: 43-66.
Westburry C, Bub D. Primary progressive aphasia: a review of 112 cases. BrainLang 1997;60:381-406.
Clark DG, Charuvastra A, Miller BL, Shapira JS, Mendez MF. Fluent versus nonfluent primary progressive aphasia: a comparison of clinical and functional neuroimaging features. BrainLang 2005;94:54-60.
Thompson CK, Cho S, Hsu CJ, et al. Dissociations Between Fluency And Agrammatism In Primary Progressive Aphasia. Aphasiology 2012; 26:20-43.
Rogalski ED, Johnson N, Weintraub S,. Mesulam M Increased Frequency of Learning Disability in Patients With Primary Progressive Aphasia and Their First-Degree Relatives. Arch Neurol2008;65:244-248.
Graff-Radford J, Josephs KA. Primary progressive aphasia and transient global amnesia. Arch Neurol2012;69:401-404.
Nitrini R, Hosogi-Senaha ML, Caramelli P. Primary Progressive Aphasia and Transient Global Amnesia. Arch Neurol2012;69:1214.
Ratnavalli E, Brayne C, Dawson K, Hodges JR. The prevalence of frontotemporal dementia. Neurology2002;58:1615-1621.
Rosso SM, Donker Kaat L, Baks T, et al. Frontotemporal dementia in The Netherlands: patient characteristics and prevalence estimates from a population-based study. Brain2003;126:2016-2022.
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.
Johnson JK, Diehl J, Mendez MF, et al. Frontotemporal lobar degeneration: demographic characteristics of 353 patients. Arch Neurol2005; 62:925-930.
Grossman M. The non-fluent/agrammatic variant of primary progressive aphasia. Lancet Neurol 2012;11:545-555.
Tan YE, Ng A, Kandiah N. Frontotemporal Dementia in Southeast Asia: a comparative study. Dement Geriatr Cogn Disord Extra 2013;3:1-9.
Sajjadi SA, Patterson K, Arnold RJ, Watson PC, Nestor PJ, Primary progressive aphasia. A tale of two syndromes and the rest. Neurology2012;78:1670-1677.
Senaha MLH, Caramelli P, Nitrini R, Charchat-Fichman H, Weekes BS. Semantic dementia without surface dyslexia. BrainLang 2006;99:42-43.
Wilson MA, Martínez-Cuitiño M. Semantic dementia without surface dyslexia in Spanish: unimpaired reading with impaired semantics. Behav Neurol 2012;25:273-284.

Publication Dates

Publication in this collection
Jan-Mar 2013

History

Received
05 Nov 2012
Accepted
15 Jan 2013

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

[1] Mesulam MM. Slowly progressive aphasia without generalized dementia. Ann Neurol 1982;11:592-598.

[2] Harciarik M, Kertesz, A. Primary progressive aphasias and their contribution to the contemporary knowledge about the brain-language relationship. Neuropsychol Rev 2011;21:271-287.

[3] Mesulam MM, Grossman M, Hillis A; Kertesz A, Weintraub S. The core and halo of primary progressive aphasia and semantic dementia. Ann Neurol2003;54(suppl 5):S11-S14.

[4] Knibb JA, Hodges JH. Semantic dementia and primary progressive aphasia: a problem of categorization. Alzheimer Dis Assoc Disord 2005; 19(suppl 1):S7-S14.

[5] Adlam ALR, Patterson K, Rogers TT, et al. Semantic dementia and fluent primary progressive aphasia: two sides of the same coin? Brain 2006;129:3066-3080.

[6] Rogaslki E, Mesulam M. An update on primary progressive aphasia. Curr Neurol Neurosci Rep 2007;7:388-392.

[7] Gorno-Tempini ML, Hillis AE, Weintraub S, et al. Classification of primary progressive aphasia and its variants. Neurology 2011;76:1006- 1014.

[8] Mesulam MM. Primary progressive aphasia. Ann Neurol2001;49:425-432.

[9] Mesulam MM. Primary progressive aphasia - a language-based dementia. New Engl J Med 2003;349:1535-1542.

[10] Gorno-Tempini ML, Dronkers NF, Rankin KP, et al. Cognition and Anatomy of three variants of primary progressive aphasia. Ann Neurol2004;55:335-346.

[11] Desgranges B, Matuszewski V, Piolino P, et al. Anatomical and functional alterations in semantic dementia: a voxel-based MRI and PET study. Neurobiol Aging 2007;28:1904-1913.

[12] Knibb JA, Xuereb JH, Patterson K, Hodges JR. Clinical and pathological characterization of progressive aphasia. Ann Neurol2006;59:156-165.

[13] Hodges JR, Mitchell J, Dawson K, et al. Semantic dementia: demography, familial factors and survival in a consecutive series of 100 cases. Brain2010;133:300-306.

[14] Grossman M. Primary progressive aphasia: clinicopathological correlations. Nat Rev Neurol 2010;6:88-97.

[15] Leyton CE, Villemagne VL, Savage S, et al. Subtypes of progressive aphasia: application of the International Consensus Criteria and validation using b-amyloid imaging. Brain2011;134:3030-3043.

[16] Nespoulous JL, Lecours AR, Lafond D, Parente MAMP. Protocole Montréal-Tolouse MT-86 d'examen linguistique de l'aphasie - version Beta. Laboratoire Théophile-Alajouanine, Montréal; 1986.

[17] Goodglass H, Kaplan E. The Assessment of aphasia and related disorders. 2nd, Philadelphia, PA, USA: Lea & Febiger; 1993.

[18] Kaplan E, Goodglass H, Weintraub S.The Boston Naming Test. Philadelphia: Lea & Febiger; 1983.

[19] Parente MAMP, Hosogi ML, Delgado AP, Lecours, AR. Protocolo de Leitura para o projeto H.F.S.P. (Human Frontier Science Program); 1992.

[20] Senaha MLH, Caramelli P, Porto CS, Nitrini R. Verbal and non-verbal semantic impairment: from fluent primary progressive aphasia to semantic dementia. Dement Neuropsychol 2007;2:203-211.

[21] Senaha MLH, Caramelli P, Porto CS, Nitrini R. Semantic dementia Brazilian study of nineteen cases. Dement Neuropsychol2007;1:366-373.

[22] Darley FL, Aronson AE, Brown JR. Motor speech disorders. Philadelphia: W.B. Saunders; 1975.

[23] Agniel A, Joanette Y, Dojon B, Duchein C. Protocole d'évaluation des gnosies visuelles. Montréal-Toulouse. Isbergues: Ortho-édition; 1992.

[24] Howard D, Patterson K. Pyramids and palm trees: a test of semantic access from pictures and words. Bury St Edmunds, Suffolk: Thames Valley Test Company; 1992.

[25] Otsuki M, Soma Y, Sato M, Homma A, Tsuji S. Slowly Progressive Pure Word Deafness. Eur Neurol 1998;39:135-140.

[26] Gorno-Tempini ML, Brambati SM, Ginex V, et al. The logopenic/phonological variant of primary progressive aphasia. Neurology2008;71:1227-1234.

[27] Henry ML,. Gorno-Tempini ML The logopenic variant of primary progressive aphasia. Curr Opin Neurol 2010;23:633-637.

[28] Rohrer JD, Rossor MN, Warren JD. Neologistic jargon aphasia and agraphia in primary progressive aphasia. J Neurol Sci 2009;277:155-159.

[29] Folstein MF, Folstein SE, Mchugh PR. Mini-mental state. A practical method for grading the cognitive state of the patients for the clinician. J Psychiatry Res 1975;12:189-198.

[30] Brucki SM, Nitrini R, Caramelli P, Bertolucci PH, Okamoto IH. Suggestions for utilization of the mini-mental state examination in Brazil. Arq Neuropsiquiatr 2003;61:777-781.

[31] Mesulam M, Wieneke C, Rogalski E, Cobia D, Thompson C, Weintraub S.Quantitative template for subtyping primary progressive aphasia. Arch Neurol 2009;66:1545-1551.

[32] Mesulam MM, Wieneke C , Thompson C, Rogalski E, Weintraub S.Quantitative classification of primary progressive aphasia at early and mild impairment stages. Brain2012;135:1537-1553.

[33] Mesulam MM, Weintraub S.Primary progressive aphasia: sharpening the focus on a clinical syndrome. In: Boller, F et al. (eds.) Heterogeneuty of Alzheimer's disease. Springer-Verlag. Berlim Heiderlberg;1992: 43-66.

[34] Westburry C, Bub D. Primary progressive aphasia: a review of 112 cases. BrainLang 1997;60:381-406.

[35] Clark DG, Charuvastra A, Miller BL, Shapira JS, Mendez MF. Fluent versus nonfluent primary progressive aphasia: a comparison of clinical and functional neuroimaging features. BrainLang 2005;94:54-60.

[36] Thompson CK, Cho S, Hsu CJ, et al. Dissociations Between Fluency And Agrammatism In Primary Progressive Aphasia. Aphasiology 2012; 26:20-43.

[37] Rogalski ED, Johnson N, Weintraub S,. Mesulam M Increased Frequency of Learning Disability in Patients With Primary Progressive Aphasia and Their First-Degree Relatives. Arch Neurol2008;65:244-248.

[38] Graff-Radford J, Josephs KA. Primary progressive aphasia and transient global amnesia. Arch Neurol2012;69:401-404.

[39] Nitrini R, Hosogi-Senaha ML, Caramelli P. Primary Progressive Aphasia and Transient Global Amnesia. Arch Neurol2012;69:1214.

[40] Ratnavalli E, Brayne C, Dawson K, Hodges JR. The prevalence of frontotemporal dementia. Neurology2002;58:1615-1621.

[41] Rosso SM, Donker Kaat L, Baks T, et al. Frontotemporal dementia in The Netherlands: patient characteristics and prevalence estimates from a population-based study. Brain2003;126:2016-2022.

[42] 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.

[43] Johnson JK, Diehl J, Mendez MF, et al. Frontotemporal lobar degeneration: demographic characteristics of 353 patients. Arch Neurol2005; 62:925-930.

[44] Grossman M. The non-fluent/agrammatic variant of primary progressive aphasia. Lancet Neurol 2012;11:545-555.

[45] Tan YE, Ng A, Kandiah N. Frontotemporal Dementia in Southeast Asia: a comparative study. Dement Geriatr Cogn Disord Extra 2013;3:1-9.

[46] Sajjadi SA, Patterson K, Arnold RJ, Watson PC, Nestor PJ, Primary progressive aphasia. A tale of two syndromes and the rest. Neurology2012;78:1670-1677.

[47] Senaha MLH, Caramelli P, Nitrini R, Charchat-Fichman H, Weekes BS. Semantic dementia without surface dyslexia. BrainLang 2006;99:42-43.

[48] Wilson MA, Martínez-Cuitiño M. Semantic dementia without surface dyslexia in Spanish: unimpaired reading with impaired semantics. Behav Neurol 2012;25:273-284.

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