Marchese, SM;
Palesi, F;
Nigri, A;
Bruzzone, MG;
Pantaleoni, C;
Gandini Wheeler-Kingshott, CAM;
D’Arrigo, S;
... Cavallari, P; + view all
(2023)
Structural and connectivity parameters reveal spared connectivity in young patients with non-progressive compared to slow-progressive cerebellar ataxia.
Frontiers in Neurology
, 14
, Article 1279616. 10.3389/fneur.2023.1279616.
Preview |
Text
fneur-14-1279616.pdf - Published Version Download (1MB) | Preview |
Abstract
INTRODUCTION: Within Pediatric Cerebellar Ataxias (PCAs), patients with non-progressive ataxia (NonP) surprisingly show postural motor behavior comparable to that of healthy controls, differently to slow-progressive ataxia patients (SlowP). This difference may depend on the building of compensatory strategies of the intact areas in NonP brain network. METHODS: Eleven PCAs patients were recruited: five with NonP and six with SlowP. We assessed volumetric and axonal bundles alterations with a multimodal approach to investigate whether eventual spared connectivity between basal ganglia and cerebellum explains the different postural motor behavior of NonP and SlowP patients. RESULTS: Cerebellar lobules were smaller in SlowP patients. NonP patients showed a lower number of streamlines in the cerebello-thalamo-cortical tracts but a generalized higher integrity of white matter tracts connecting the cortex and the basal ganglia with the cerebellum. DISCUSSION: This work reveals that the axonal bundles connecting the cerebellum with basal ganglia and cortex demonstrate a higher integrity in NonP patients. This evidence highlights the importance of the cerebellum-basal ganglia connectivity to explain the different postural motor behavior of NonP and SlowP patients and support the possible compensatory role of basal ganglia in patients with stable cerebellar malformation.
| Type: | Article |
|---|---|
| Title: | Structural and connectivity parameters reveal spared connectivity in young patients with non-progressive compared to slow-progressive cerebellar ataxia |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.3389/fneur.2023.1279616 |
| Publisher version: | https://doi.org/10.3389/fneur.2023.1279616 |
| Language: | English |
| Additional information: | © 2023 Marchese, Palesi, Nigri, Bruzzone, Pantaleoni, Gandini Wheeler-Kingshott, D’Arrigo, D’Angelo and Cavallari. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
| Keywords: | Cerebellar hypoplasia, cerebellar atrophy, MRI, white matter, gray matter, cerebrocerebellar loops, basal ganglia |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Neuroinflammation |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10183206 |
Archive Staff Only
 |
View Item |
