Primary progressive apraxia of speech (PPAOS) is a clinical syndrome in
which apraxia of speech is the initial indication of neurodegenerative disease.
Prior studies of PPAOS have identified hypometabolism, grey matter atrophy, and
white matter tract degeneration in the frontal gyri, precentral cortex, and
supplementary motor area (SMA). Recent clinical observations suggest two
distinct subtypes of PPAOS may exist. Phonetic PPAOS is characterized
predominantly by distorted sound substitutions. Prosodic PPAOS is characterized
predominantly by slow, segmented speech. Demographic, clinical, and neuroimaging
data (MRI, DTI, and FDG-PET) were analyzed to validate these subtypes and
explore anatomic correlates. The Phonetic subtype demonstrated bilateral
involvement of the SMA, precentral gyrus, and cerebellar crus. The Prosodic
subtype demonstrated more focal involvement in the SMA and right superior
cerebellar peduncle. The findings provide converging evidence that differences
in the reliably determined predominant clinical characteristics of AOS are
associated with distinct imaging patterns, independent of severity.