Primary progressive aphasia: classification of variants in 100 consecutive Brazilian cases.

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

doi:10.1590/s1980-57642013dn70100017

Mirna Lie Hosogi Senaha, Ricardo Nitrini + Show 9 more

Open Access

https://doi.org/10.1590/s1980-57642013dn70100017

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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).OBJECTIVETo analyze the demographic data and the clinical classification of 100 PPA cases.METHODSData 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.RESULTSThe 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.CONCLUSIONIt 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.

Highlights

Primary progressive aphasia (PPA) is a neurodegenerative clinical syndrome characterized primarily by progressive language impairment
Ninety-eight patients were right-handed, one case had a history of developmental dyslexia and two cases had a history of transient global amnesia episodes before the emergence of language disturbance
The data reported in this study included a large number of consecutive PPA cases submitted to language evaluation by the same speech pathologist over a period of 13 years

Summary

Introduction

Primary progressive aphasia (PPA) is a neurodegenerative clinical syndrome characterized primarily by progressive language impairment. Systematic studies on PPA started after Mesulam[1] published his seminal paper entitled “Slowly progressive aphasia without generalized dementia” in 1982. Cases reported over the past 100 years by Pick, Déjerine, Sérieux and Rosenfeld, which presented degenerative diseases with language disturbances in the initial phase, would possibly fit current criteria for the condition recognized as PPA.[2]. There have been different visions and controversies about the classification and diagnosis of this syndrome.[3,4,5,6] more recently, consensus diagnostic criteria were published with the objective of providing a standard approach to the diagnosis of PPA and its classification across multicenter studies, by a group of experienced clinicians and researchers from different centers.[7]

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