Primary Progressive Aphasia in Northeast Brazil

Brief History

In 1892, Arnold Pick first described a progressive language disorder. In this disorder, there was atrophy in the frontal and temporal regions of the left cerebral hemisphere which he called progressive non-fluent or agrammatic aphasia (Hopper et al. 2023). A century later, Mesulam carefully studied six right-handed patients with slowly progressive aphasia, without intellectual and behavioral disorders. In most of these cases, symptoms began in the pre-senile phase due to anomic aphasia in 5 patients, and speech deafness in 1 of them. In the 90s, Neary et al. published the criteria for frontotemporal lobar degeneration. Based on these criteria, it was observed that lesions affecting the right side of the brain had different clinical expressions than those in the left hemisphere. On the right, behavioral variant frontotemporal dementia (bv-FTD) stood out, while on the left, PPA. An impairment in the left frontotemporal region can give rise to two distinct forms of PPA: the non-fluent variant, and the semantic variant (Kertesz et al. 1999). In 2001, in Northeast Brazil, Brito-Marques & Vieira-Mello described a case of non-fluent variant PPA in a patient with a pathological diagnosis of Pick’s disease (Figure 1). This was the first case of PPA published in Brazil with pathological confirmation.

Figure 1.

Shows the upper left MRI image, showing significant left frontal, temporal, and parietal atrophy; on the right, upper functional neuroimaging of significant hypoperfusion in the frontal region, especially on the right; on the lower left, macroscopic image of the brain with moderate atrophy in the frontal region; and on the lower right the presence of Pick's corpuscles (Professor Paulo Brito Collection)

New studies were carried out in 2004 by Gorno-Tempini et al., describing the third variant of PPA, the logopenic variant of primary progressive aphasia (lv-PPA). After the three APP variants were classified, their frequency was described in 2006 by Amici et al. This group carried out a clinical, neuropsychological, and neuroimaging study showing that the most common variants of PPA in regressive order are: 1. Nonfluent variant primary progressive aphasia (nfv-PPA), 2. Primary progressive aphasia semantic variant (sv-PPA), and 3. Logopenic variant primary progressive aphasia (lv-PPA). With APP advances its complexity has also increased, to better study, in 2009, Rogalski et al. proposed the term mild cognitive impairment (MCI) of PPA, a specific designation for the border zone between normal aging and amnestic dementia and PPA. To organize the new APP classification, in 2011, Gorno-Tempini et al. published the clinical criteria for diagnosing PPA and its 3 main variants. In principle, they proposed that patients be diagnosed with PPA and then divided into subtypes according to the language characteristics of each subtype, in addition to supporting neuroimaging and genetic studies. In 2011, after updating the new criteria for PPA, in Northeast Brazil, Brito-Marques et al. published the second case of APP in Brazil with pathological confirmation. It was a case of nfv-PPA in a patient with corticobasal degeneration, whose clinical picture predominated with changes in executive functions, in praxis, and mainly in the language (Figure 2).

Figure 2.

Shows upper left structural neuroimaging with significant frontoparietal atrophy, especially parietal; on the upper right, functional neuroimaging shows severe frontal hypoperfusion; the lower left shows a macroscopic image with moderate bilateral frontal atrophy; and on the lower right, an image of balloon cells is seen (Professor Paulo Brito Collection)