A - 87 A Neuropsychological Case Study of Primary Progressive Apraxia of Speech Versus Agrammatic Primary Progressive Aphasia

Abstract Objective Primary progressive apraxia of speech (PPAOS) is a lesser-known neurodegenerative disorder characterized by progressive speech production loss, affecting word articulation. Apraxic speech commonly occurs in primary progressive aphasias (PPAs) and as a result, is often misdiagnosed as agrammatic PPA (aPPA). The current case highlights the importance of neuropsychological evaluations and behavioral observations in delineating PPAOS from aPPA. Method The patient is a 71-year-old right-handed White female who presented with a 2-year history of language difficulties beginning with a lisp and progressing to an inability to produce words. MRI of the brain showed moderate white matter disease. Results Her neurocognitive profile was notable for primary deficits in expressive language, including oral/written production, verbal fluency, and repetition. Milder deficits were observed in aspects of executive functioning. Observed spontaneous speech was notable for impaired and effortful articulation with syllabically segmented prosodic speech patterns, some distortions and prosody alterations, and slow rate. All other cognitive domains were intact. Conclusions Patients with PPAOS and aPPA typically score in the impaired range on tests that depend on rate and accuracy of speech. Apraxia was her most salient feature reported and observed on testing. We did not find learning and memory difficulties, which are more common in aPPA. Moreover, she had trial-and-error articulatory attempts, groping, and distorted substitutions, which are more commonly seen in PPAOS. Her speech was also devoid of grammatical errors which is another distinguishing feature. While subtle and confounded by her alcohol use and orthopedic injuries, her profile altogether appeared most consistent with PPAOS.