DOI: 10.1101/19007831Oct 8, 2019Paper

Frontal and cerebellar atrophy supports FTLD-ALS clinical continuum and neuropsychology

MedRxiv : the Preprint Server for Health Sciences
Beatrice PizzarottiC. A. M. Gandini Wheeler-Kingshott

Abstract

Objective: Frontotemporal Lobe Degeneration (FTLD) and Amyotrophic Lateral Sclerosis (ALS) are neurodegenerative diseases more often considered as a continuum from clinical, epidemiologic and genetic perspectives. We used localized brain atrophy to evaluate common and specific features of FTLD, FTLD-ALS and ALS patients to clarify this clinical continuum. Methods: We used voxel-based morphometry (VBM) on structural MRI images to localize volume alterations of brain regions in group comparisons: patients (20 FTLD, 7 FTLD-ALS, 18 ALS) versus controls (39 CTR) and patient groups between themselves. We used whole-brain cortical thickness (CT) to assess correlations with brain volume to propose mechanistic explanations of the heterogeneous clinical presentations. We assessed whether brain atrophy can explain cognitive impairment, measured with neuropsychological tests (Frontal Assessment Battery, verbal fluency and semantic fluency). Results: Common (mainly frontal) and specific areas of atrophy between FTLD, FTLD-ALS and ALS patients were detected, on the one hand confirming the suggestion of a clinical continuum, while on the other hand defining morphological specificities for each clinical group (e.g. a different cerebral and cer...Continue Reading

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