Unveiling the enigmatic: Primary progressive apraxia of speech – A case report

Abstract

Primary progressive apraxia of speech (PPAOS) is a rare neurodegenerative disorder that saliently affects motor speech programming and planning. Linguistic function remains intact in the early stages of PPAOS.

Although PPAOS shares a similar symptomatology to conditions such as primary progressive aphasia (PPA) and dysarthria, it is important to remember that this disorder constitutes its own distinct clinical syndrome. PPAOS is characterized by an individually variable disease course, with a steady progression in speech deterioration. In later stages, this disorder may additionally present with symptoms such as oral apraxia, dysarthria, dysphagia, aphasia, and parkinsonian syndromes similar to either progressive supranuclear palsy (PSP) or corticobasal syndrome (CBS). 4-repeat tauopathy is the most common pathology associated with PPAOS. In this study, we present a case of a female patient suffering from PPAOS, detailing her clinical course during a 44-year long follow-up. As PPAOS is a disorder with a worldwide poorly-documented prevalence, there is limited data in literature on the subject. We thus bring this case to public discussion. We also recommend further investigating this disorder, as we would then be able to unify diagnostic and therapeutic approaches for PPAOS.