Abstract

Progressive non-fluent aphasia (PNFA) is a neurodegenerative disorder characterized by agrammatism and apraxia of speech. Other features include impaired comprehension of syntactically complex structures, intact object knowledge, and spared single-word comprehension. Given the complexity of primary progressive aphasia (PPA) syndromes, neuropsychologists can play a vital role in differential diagnosis, particularly when there are comorbid medical factors. This case study presents on a 74-year-old female with progressive language and cognitive impairment over the past 8years whose care was jointly managed in specialty neurology clinics (e.g., Epilepsy and Memory Disorders) at a university medical center. She had a history of generalized convulsive seizures but with none occurring in several years. She was weaned off phenobarbital over the course of a few months to help rule out any adverse effects on functioning but her deficits persisted. Follow-up MRI showed mild-to-moderate diffuse atrophy. Her cognitive neurologist raised concerns for PPA which prompted the neuropsychological evaluation 3-4months later. Testing showed prominent impairments in fluency, naming, repetition, oral reading, and comprehension for grammatically complex questions while semantic knowledge for objects was preserved. Conversational language was halting, effortful, dysfluent, and anomic. Broader cognitive deficits were also observed during testing which was suggestive of more generalized cerebral dysfunction. The patient's profile was highly concerning for PNFA. Further work-up at the institution's Frontotemporal Dementia Clinic along with an FDG-PET scan were recommended to help further clarify her profile. Overall, this case highlights the utility of neuropsychology in the differential diagnosis of complex neurodegenerative disorders with comorbid medical factors.

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