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ABSTRACT

Objective: To report a case of Frontotemporal Dementia Variant Primary Progressive Aphasia with agrammatism (FTD-PPA), a rare neurodegenerative disease that represents significant challenges in differential diagnosis, particularly with Alzheimer’s Disease (AD). Case detail: A 62-year-old female initially presented with amnesia, anxiety, and speech difficulties, managed with fluoxetine, resulting in partial improvement. Over three years, she exhibited progressive cognitive and functional decline, including mental confusion, aphasia, motor difficulties, and significant weight loss. Mini-Mental State Examination scores deteriorated from 20/30 to 9/30. The diagnosis was established by prioritizing the decisive cerebrospinal fluid biomarker evidence, which excluded AD, and the dominant clinical phenotype of progressive aphasia over the atypical neuroimaging findings. Management included antidepressants, donepezil (later discontinued), and speech therapy, with limited cognitive improvement despite mood stabilization. Final considerations: This case highlights the complexities of diagnosing progressive cognitive impairment. Comprehensive clinical, neuropsychological, and biomarker evaluations were critical in differentiating FTD-PPA from AD. The predominance of linguistic deficits and biomarker results confirmed the diagnosis of the agrammatic variant of FTD-PPA. Multidisciplinary follow-up and individualized symptom management were essential in optimizing the patient’s quality of life.

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