Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2023 Nov;83(7):665-673.
doi: 10.1002/jdn.10295. Epub 2023 Aug 21.

Brain structural changes in alternating hemiplegia of childhood using single-case voxel-based morphometry analysis

Elly Arizono  1   2 , Noriko Sato  1 , Yoko Shigemoto  1 , Yukio Kimura  1 , Emiko Chiba  1 , Hiroyuki Maki  1 , Hiroshi Matsuda  3 , Eri Takeshita  4 , Yuko Shimizu-Motohashi  4 , Masayuki Sasaki  4 , Kazuhiro Saito  2
Affiliations

Brain structural changes in alternating hemiplegia of childhood using single-case voxel-based morphometry analysis

Elly Arizono et al. Int J Dev Neurosci. 2023 Nov.

Abstract

Background and purpose: Alternating hemiplegia of childhood (AHC) is a rare neurodevelopmental disease caused by ATP1A3 mutations. Using voxel-based morphometry (VBM) analysis, we compared an AHC patient cohort with controls. Additionally, with single-case VBM analysis, we assessed the associations between clinical severity and brain volume in patients with AHC.

Materials and methods: To investigate structural brain changes in gray matter (GM) and white matter (WM) volumes between 9 patients with AHC and 20 age-matched controls, VBM analysis was performed using three-dimensional T1-weighted magnetic resonance imaging. Single-case VBM analysis was also performed on nine patients with AHC to investigate the associations between the respective volumes of GM/WM differences and the motor level, cognitive level, and status epilepticus severity in patients with AHC.

Results: Compared with controls, patients with AHC showed significant GM volume reductions in both hippocampi and diffuse cerebellum, and there were WM reductions in both cerebral hemispheres. In patients with AHC, cases with more motor dysfunction, the less GM/WM volume of cerebellum was shown. Three of the six cases with cognitive dysfunction showed a clear GM volume reduction in the insulae. Five of the six cases with status epilepticus showed the GM volume reduction in hippocampi. One case had severe status epilepticus without motor dysfunction and showed no cerebellar atrophy.

Conclusion: With single-case VBM analysis, we could show the association between region-specific changes in brain volume and the severity of various clinical symptoms even in a small sample of subjects.

Keywords: ATP1A3; MRI; alternating hemiplegia of childhood; cerebellum; single-case voxel-based morphometry.

PubMed Disclaimer

References

REFERENCES

    1. Ashburner, J. (2007). A fast diffeomorphic image registration algorithm. NeuroImage, 38, 95-113. https://doi.org/10.1016/j.neuroimage.2007.07.007
    1. Ashburner, J., & Friston, K. J. (2000). Voxel-based morphometry-The methods. NeuroImage, 11, 805-821. https://doi.org/10.1006/nimg.2000.0582
    1. Cartagena, A. M., Young, G. B., Lee, D. H., & Mirsattari, S. M. (2014). Reversible and irreversible cranial MRI findings associated with status epilepticus. Epilepsy & Behavior, 33, 24-30. https://doi.org/10.1016/j.yebeh.2014.02.003
    1. Chang, L. J., Yarkoni, T., Khaw, M. W., & Sanfey, A. G. (2013). Decoding the role of the insula in human cognition: Functional parcellation and large-scale reverse inference. Cerebral Cortex, 23, 739-749. https://doi.org/10.1093/cercor/bhs065
    1. Chu, Y., Parada, I., & Prince, D. A. (2009). Temporal and topographic alterations in expression of the α3 isoform of Na+, K+-ATPase in the rat freeze lesion model of microgyria and epileptogenesis. Neuroscience, 162, 339-348. https://doi.org/10.1016/j.neuroscience.2009.04.003

Substances

Supplementary concepts

LinkOut - more resources