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AJNR Am J Neuroradiol. 2019 Nov 14. doi: 10.3174/ajnr.A6306. [Epub ahead of print]

Topological Alterations of the Structural Brain Connectivity Network in Children with Juvenile Neuronal Ceroid Lipofuscinosis.

Author information

1
From the Hospital District of Helsinki and Uusimaa Medical Imaging Center (T.R., U.R., A.T., M.H.B., T.A.) timo.roine@iki.fi.
2
Turku Brain and Mind Center (T.R.), University of Turku, Turku, Finland.
3
Department of Neuroscience and Biomedical Engineering (T.R.), Aalto University School of Science, Espoo, Finland.
4
From the Hospital District of Helsinki and Uusimaa Medical Imaging Center (T.R., U.R., A.T., M.H.B., T.A.).
5
Radiology, Child Psychiatry (M.M.).
6
Department of Psychiatry (L.Å.), University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
7
Department of Child Neurology (T.L.), Children's Hospital, University of Helsinki and Helsinki University, Helsinki, Finland.

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.

PMID:
31727742
DOI:
10.3174/ajnr.A6306

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