Primary progressive aphasia (PPA) is a progressive neurodegenerative syndrome characterized by selective language impairment and is described as semantic, logopenic or nonfluent agrammatic variant. 14-3-3, a regulatory neuronal protein, has been known to be associated with CJD, Parkinson Disease, and Alzheimer Disease.

Here we discuss a case of a 65 year-old right-handed male who presents for evaluation of gradual progressive decline in speech over a 6-month period that acutely worsened within the 2 weeks prior to presentation. On physical exam he was unable to organize language and had frequent paraphasias and consistent circumlocutions. There was significant impairment in sentence repetition and single word retrieval. These symptoms were rapid and severe; in those short 6 months, the patient had relinquished his duties as a Chief Executive Officer of his company.

MRI-brain revealed left parietal, temporal, and occipital atrophy, but uniquely demonstrated leptomeningeal enhancement with vasogenic edema in the same area. Biopsy of a right frontal lesion showed beta-amyloid plaques within the cortex. Together with the clinical picture, the diagnosis of logopenic variant PPA was made.

Given his uncommon presentation, additional laboratory testing was performed and revealed his CSF was negative for RT-QuIC and T-tau was 350 (Non-Prion disease 0-1149), however, 14-3-3 was positive. He was started on Namenda and intensive speech therapy with significant improvement in speech and cognitive functions.