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Abstract
Progressive apraxia of speech is a neurodegenerative syndrome affecting spoken communication. Molecular pathology, biochemistry, genetics, and longitudinal imaging were investigated in 32 autopsy-confirmed patients with progressive apraxia of speech who were followed over 10 years. Corticobasal degeneration and progressive supranuclear palsy (4R-tauopathies) were the most common underlying pathologies. Perceptually distinct speech characteristics, combined with age-at-onset, predicted specific 4R-tauopathy; phonetic subtype and younger age predicted corticobasal degeneration, and prosodic subtype and older age predicted progressive supranuclear palsy. Phonetic and prosodic subtypes showed differing relationships within the cortico-striato-pallido-nigro-luysial network. Biochemical analysis revealed no distinct differences in aggregated 4R-tau while tau H1 haplotype frequency (69%) was lower compared to 1000+ autopsy-confirmed 4R-tauopathies. Corticobasal degeneration patients had faster rates of decline, greater cortical degeneration, and shorter illness duration than progressive supranuclear palsy. These findings help define the pathobiology of progressive apraxia of speech and may have consequences for development of 4R-tau targeting treatment.
Highlights
Progressive apraxia of speech is a neurodegenerative syndrome affecting spoken communication
Of the 32 progressive AOS (PAOS) patients, the most common underlying pathology was corticobasal degeneration (CBD), which was found in 17 cases (53%), followed by progressive supranuclear palsy (PSP), which was observed in 10 cases (31%, Fig. 1)
We found that PAOS is most often associated with a 4R tauopathy when it exists in isolation or when it co-exists with aphasia
Summary
Progressive apraxia of speech is a neurodegenerative syndrome affecting spoken communication. Corticobasal degeneration patients had faster rates of decline, greater cortical degeneration, and shorter illness duration than progressive supranuclear palsy. These findings help define the pathobiology of progressive apraxia of speech and may have consequences for development of 4R-tau targeting treatment. In the past two decades, a subtype of AOS has been described that is insidious in onset and progressive in nature[4,5,6] This progressive AOS (PAOS) often accompanies progressive agrammatic aphasia[7] (AOS-PAA) or occurs as an embedded feature of a more widespread neurodegenerative syndrome, such as corticobasal syndrome (CBS) or amyotrophic lateral sclerosis[8] (referred to here as +AOS).
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