Clinical and Linguistic Profiles and Challenges in Diagnosis of Primary Progressive Aphasia in Medan, Indonesia: A Hospital-based Study

Abstract

Primary Progressive Aphasia (PPA) is a degenerative condition characterized by progressive loss of language function. Studies on PPA patients in Indonesia are still limited, and none has reported clinical and linguistic profiles of PPA patients who speak Bahasa Indonesia. This study aimed to describe clinical and linguistic profiles and challenges in the diagnosis of PPA patients from referral hospitals in Medan, Indonesia. We retrospectively reviewed the clinical records of patients diagnosed with PPA based on the 2011 diagnostic criteria during the 2022-2023 period and described clinical characteristics data and linguistic profiles using descriptive analysis. We included 6 cases that fulfilled the diagnostic criteria for PPA. There were 3 cases categorized as nfvPPA, 1 case as svPPA, and 2 cases as lvPPA. There was female predominance (83.3%) and the mean age at onset was 59±2.96 years. The first symptom reported in the nfvPPA group was effortful, non-fluent speech; in svPPA, it was impaired naming and single word comprehension, while in lvPPA, it was impairment in word retrieval. Challenges in diagnosis included the availability of a standardized language tool aimed specifically for PPA in Bahasa Indonesia and the expertise needed to make such a diagnosis. The main clinical features of the PPA reported were similar to previous findings with specific characteristics of Bahasa Indonesia. Determining language profiles of each variant of PPA in Bahasa Indonesia is crucial to establishing a correct diagnosis. Language assessment tool in Bahasa Indonesia is urgently needed to facilitate better assessment and management planning to improve quality of life.