A1 Refereed original research article in a scientific journal
Topological Alterations of the Structural Brain Connectivity Network in Children with Juvenile Neuronal Ceroid Lipofuscinosis
Authors: T. Roine, U. Roine, A.Tokola, M.H.Balk, M. Mannerkoski, L.Åberg, T.Lönnqvist, T. Autti
Publisher: AMER SOC NEURORADIOLOGY
Publication year: 2019
Journal: American Journal of Neuroradiology
Journal name in source: AMERICAN JOURNAL OF NEURORADIOLOGY
Journal acronym: AM J NEURORADIOL
Volume: 40
Issue: 12
First page : 2146
Last page: 2153
Number of pages: 8
ISSN: 0195-6108
eISSN: 1936-959X
DOI: https://doi.org/10.3174/ajnr.A6306
Abstract
BACKGROUND AND PURPOSE: We used diffusion MR imaging to investigate the structural brain connectivity networks in juvenile neuronal ceroid lipofuscinosis, a neurodegenerative lysosomal storage disease of childhood. Although changes in conventional MR imaging are typically not visually apparent in children aged <10 years, we previously found significant microstructural abnormalities by using diffusion MR imaging. Therefore, we hypothesized that the structural connectivity networks would also be affected in the disease. MATERIALS AND METHODS: We acquired diffusion MR imaging data from 14 children with juvenile neuronal ceroid lipofuscinosis (mean ? SD age, 9.6 ? 3.4?years; 10 boys) and 14 control subjects (mean ? SD age, 11.2 ? 2.3?years; 7 boys). A follow-up MR imaging was performed for 12 of the patients (mean ? SD age, 11.4 ? 3.2 years; 8 boys). We used graph theoretical analysis to investigate the global and local properties of the structural brain connectivity networks reconstructed with constrained spherical deconvolution?based whole-brain probabilistic tractography. RESULTS: We found significantly increased characteristic path length (P?=?.003) and decreased degree (P?=?.003), which indicated decreased network integration and centrality in children with juvenile neuronal ceroid lipofuscinosis. The findings were similar for the follow-up MR imaging, and there were no significant differences between the two acquisitions of the patients. In addition, we found that the disease severity correlated negatively (P< .007) with integration, segregation, centrality, and small-worldness of the networks. Moreover, we found significantly (P < .0003) decreased local efficiency in the left supramarginal gyrus and temporal plane, and decreased strength in the right lingual gyrus.CONCLUSIONS: We found significant global and local network alterations in juvenile neuronal ceroid lipofuscinosis that correlated with the disease severity and in areas related to the symptomatology.
BACKGROUND AND PURPOSE: We used diffusion MR imaging to investigate the structural brain connectivity networks in juvenile neuronal ceroid lipofuscinosis, a neurodegenerative lysosomal storage disease of childhood. Although changes in conventional MR imaging are typically not visually apparent in children aged <10 years, we previously found significant microstructural abnormalities by using diffusion MR imaging. Therefore, we hypothesized that the structural connectivity networks would also be affected in the disease. MATERIALS AND METHODS: We acquired diffusion MR imaging data from 14 children with juvenile neuronal ceroid lipofuscinosis (mean ? SD age, 9.6 ? 3.4?years; 10 boys) and 14 control subjects (mean ? SD age, 11.2 ? 2.3?years; 7 boys). A follow-up MR imaging was performed for 12 of the patients (mean ? SD age, 11.4 ? 3.2 years; 8 boys). We used graph theoretical analysis to investigate the global and local properties of the structural brain connectivity networks reconstructed with constrained spherical deconvolution?based whole-brain probabilistic tractography. RESULTS: We found significantly increased characteristic path length (P?=?.003) and decreased degree (P?=?.003), which indicated decreased network integration and centrality in children with juvenile neuronal ceroid lipofuscinosis. The findings were similar for the follow-up MR imaging, and there were no significant differences between the two acquisitions of the patients. In addition, we found that the disease severity correlated negatively (P< .007) with integration, segregation, centrality, and small-worldness of the networks. Moreover, we found significantly (P < .0003) decreased local efficiency in the left supramarginal gyrus and temporal plane, and decreased strength in the right lingual gyrus.CONCLUSIONS: We found significant global and local network alterations in juvenile neuronal ceroid lipofuscinosis that correlated with the disease severity and in areas related to the symptomatology.
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