Clinical profile of primary progressive Aphasia in Peru: Case series from a Neurological care center

Abstract

AbstractBackgroundPrimary Progressive Aphasia (PPA) is one of the clinical syndromes of frontotemporal dementia (1). The frequency of PPA syndromes is low (16%) in this Latin America, with just one published report in Peruvian population (2). We aim to describe clinical features of PPA patients followed up at a Neurological healthcare center in Peru.MethodRetrospective review of clinical records of patients diagnosed with probable PPA (based on 2011 diagnostic criteria) during the 2010‐2020 period. Local IRB approval was obtained for this study.ResultWe included 11 cases that fulfilled the selection criteria. Nine cases (75%) were categorized as nfvPPA, and 3 (25%) svPPA. There was slightly female predominance (58%). The average of education was 10.8±4.8 years. Six cases (54.5%) declared place of birth from northern Peru. We found 4.2±1.8 years of diagnostic delay for PPA. The mean age at onset for nfvPPA and svPPA groups was 63 ±12.4, and 52(52‐64) respectively. The first symptom identified in the nfvPPA group was effortful, halting speech 7 (77.8%), while svPPA was impaired confrontation naming 3 (100%). nfvPPA group showed agrammatism in language production and effortful speech in 8 patients (88.9%) and impaired in comprehension of syntactically complex sentences and spared single ‐word comprehension 3(33.3%). Parkinsonism was present in 6 patients (66.7%) and only one patient reported coexistence of amyotrophic lateral sclerosis. Depression was present in most of the 50% of cases. svPPA group also presented impaired in single word‐ comprehension, in object knowledge, and spared repetition 3(100%), 3 (100%) and 2(66.8%) respectively. Less than 50% of PPA cases were in speech therapy.ConclusionThe main clinical features of the PPA reported are mostly consistent with previous reports. In our medical records review, no cases of apraxia of speech were found. There is a significant delay of PPA diagnosis. Further studies with prospective design are required to obtain a better description. Implementing training strategies for clinicians will allow to improve the timely and accurate diagnosis of PPA in Peru.