Behavioral variant frontotemporal dementia in the setting of progressive apraxia of speech: A clinico-neuroimaging case-control study

Title : Behavioral variant frontotemporal dementia in the setting of progressive apraxia of speech: A clinico-neuroimaging case-control study

Abstract:

Objective: Progressive apraxia of speech (PAOS) is a neurodegenerative syndrome related to the propositional and schematic aspects of motor speech production, whereas behavioral variant frontotemporal dementia (bvFTD) is due to a decline in behavioral features. Clinical and anatomic characteristics of bvFTD in the setting of PAOS is understudied. 
Methods: Among 66 prospectively recruited PAOS participants, 12 were identified as having features consistent with or reminiscent of bvFTD. Eleven of these PAOS-bvFTD participants completed a volumetric head MRI scan. We compared clinical and anatomical atrophy patterns of these 11 PAOS-bvFTD participants to 11 PAOS participants without bvFTD and 22 healthy controls. Statistical Parametric Mapping (SPM) was applied using BrainRenders to visualize gray matter volume across both groups compared to controls and to each other. 
Results: Those with PAOS-bvFTD performed worse on the Frontal Behavioral Inventory (median: 33/72 vs 10/72), Neuropsychiatry inventory (4/36 vs. 1.5/36) and 20-item Behavioral Assessment scale (4.5/20 vs. 1.0/20) compared PAOS, p<0.01. On SPM, PAOS-bvFTD and PAOS had more atrophy in frontal lobe regions compared to controls. PAOS-bvFTD showed more prefrontal atrophy than PAOS on direct comparison. 
Conclusion: Behavioral features consistent with bvFTD in PAOS is related to greater atrophy of the prefrontal cortex.

Biography:

Nadia Hossain is a physician from Bangladesh who completed her medical education and a one-year clinical internship at Dhaka Medical College, Bangladesh, in 2022. She received her post-graduation medical education certification in Clinical trials in 2024 from Harvard Medical School. She then joined Dr. Josephs’s lab in September 2024 as a postdoctoral research fellow in the Behavioral Neurology Department at the Mayo Clinic, Rochester. Her research focuses are primary progressive apraxia of speech, behavioral variant frontotemporal dementia, progressive supranuclear palsy, corticobasal degeneration, and atypical parkinsonism.