Motor neuron dysfunctions (MNDys) in Frontotemporal Lobar Degeneration (FTLD) have been consistently reported. Clinical and neurophysiological findings proved a variable range of pathological changes, also affecting the corticospinal tract (CST). This study aims to assess white-matter microstructural alterations in a sample of patients with FTLD, and to evaluate the relationship with MNDys. Fifty-four FTLD patients (21 bvFTD, 16 PPA, 17 CBS) and 36 healthy controls participated in a Diffusion Tensor Imaging (DTI) study. We analyzed distinctive and common microstructural alteration patterns across FTLD subtypes, including those affecting the CST, and performed an association analysis between CST integrity and the presence of clinical and/or neurophysiological signs of MNDys. The majority of FTLD patients showed microstructural changes in the motor pathway with a high prevalence of CST alterations also in patients not displaying clinical and/or neurophysiological signs of MNDys. Our results suggest that subtle CST alterations characterize FTLD patients regardless to the subtype. This may be due to the spread of the pathological process to the motor system, even without a clear clinical manifestation of MNDys.

Diffusion tensor imaging evidence of corticospinal pathway involvement in frontotemporal lobar degeneration

Crespi, Chiara;Cappa, Stefano F;Cerami, Chiara
2019

Abstract

Motor neuron dysfunctions (MNDys) in Frontotemporal Lobar Degeneration (FTLD) have been consistently reported. Clinical and neurophysiological findings proved a variable range of pathological changes, also affecting the corticospinal tract (CST). This study aims to assess white-matter microstructural alterations in a sample of patients with FTLD, and to evaluate the relationship with MNDys. Fifty-four FTLD patients (21 bvFTD, 16 PPA, 17 CBS) and 36 healthy controls participated in a Diffusion Tensor Imaging (DTI) study. We analyzed distinctive and common microstructural alteration patterns across FTLD subtypes, including those affecting the CST, and performed an association analysis between CST integrity and the presence of clinical and/or neurophysiological signs of MNDys. The majority of FTLD patients showed microstructural changes in the motor pathway with a high prevalence of CST alterations also in patients not displaying clinical and/or neurophysiological signs of MNDys. Our results suggest that subtle CST alterations characterize FTLD patients regardless to the subtype. This may be due to the spread of the pathological process to the motor system, even without a clear clinical manifestation of MNDys.
Corticospinal tract; Diffusion tensor imaging; FTD-ALS disease continuum; Frontotemporal lobar degeneration; Motor neuron dysfunction
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/20.500.12076/6155
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