A weighted cranial diffusion-weighted imaging scale for Wilson's disease

Shi-Jing Wang; Hao Geng; Si-Rui Cheng; Chen-Chen Xu; Rui-Qi Zhang; Yu Wang; Tong Wu; Bo Li; Tao Wang; Yong-Sheng Han; Zeng-Hui Ding; Yi-Ning Sun; Xun Wang; Yong-Zhu Han; Nan Cheng

doi:10.3389/fnins.2023.1186053

A weighted cranial diffusion-weighted imaging scale for Wilson's disease

Front Neurosci. 2023 Aug 15:17:1186053. doi: 10.3389/fnins.2023.1186053. eCollection 2023.

Authors

Shi-Jing Wang^{1

2}, Hao Geng^{3

4}, Si-Rui Cheng⁵, Chen-Chen Xu², Rui-Qi Zhang^{3

4}, Yu Wang^{3

4}, Tong Wu¹, Bo Li², Tao Wang³, Yong-Sheng Han², Zeng-Hui Ding³, Yi-Ning Sun³, Xun Wang^{1

2}, Yong-Zhu Han^{1

2}, Nan Cheng^{1

2}

Affiliations

¹ Graduate School, Anhui University of Chinese Medicine, Hefei, China.
² Hospital Affiliated to the Institute of Neurology, Anhui University of Chinese Medicine, Hefei, China.
³ Institute of Intelligent Machines, Hefei Institute of Physical Science, Chinese Academy of Sciences, Hefei, China.
⁴ Department of Biophysics, University of Science and Technology of China, Hefei, China.
⁵ Department of Economics, Nankai University, Tainjin, China.

Abstract

Objectives: Cranial magnetic resonance imaging (MRI) could be a crucial tool for the assessment for neurological symptoms in patients with Wilson's disease (WD). Diffusion-weighted imaging (DWI) hyperintensity reflects the acute brain injuries, which mainly occur in specific brain regions. Therefore, this study aimed to develop a weighted cranial DWI scale for patients with WD, with special focus on specific brain regions.

Materials and methods: In total, 123 patients with WD were enrolled, 118 of whom underwent 1.5 T-MRI on admission. The imaging score was calculated as described previously and depended on the following sequences: one point was acquired when abnormal intensity occurred in the T1, T2, and fluid-attenuation inversion recovery sequences, and two points were acquired when DWI hyperintensity were found. Consensus weighting was conducted based on the symptoms and response to treatment.

Results: Intra-rater agreement were good (r = 0.855 [0.798-0.897], p < 0.0001). DWI hyperintensity in the putamen was a high-risk factor for deterioration during de-copper therapy (OR = 8.656, p < 0.05). The high-risk factors for readmission for intravenous de-copper therapies were DWI hyperintensity in the midbrain (OR = 3.818, p < 0.05) and the corpus callosum (OR = 2.654, p < 0.05). Both scoring systems had positive correlation with UWDRS scale (original semi-quantitative scoring system, r = 0.35, p < 0.001; consensus semi-quantitative scoring system, r = 0.351, p < 0.001.). Compared to the original scoring system, the consensus scoring system had higher correlations with the occurrence of deterioration (OR = 1.052, 95%CI [1.003, 1.0103], p < 0.05) and readmission for intravenous de-copper therapy (OR = 1.043, 95%CI [1.001, 1.086], p < 0.05).

Conclusion: The predictive performance of the consensus semi-quantitative scoring system for cranial MRI was improved to guide medication, healthcare management, and prognosis prediction in patients with WD. For every point increase in the neuroimaging score, the risk of exacerbations during treatment increased by 5.2%, and the risk of readmission to the hospital within 6 months increased by 4.3%.

Keywords: MRI; Wilson disease; clinical assessment; neuroimaging; rating scores.