Stage division of Wilson's disease based on imaging indexes / 中华神经医学杂志

ABSTRACT

Objective:To explore the feasibility of staging Wilson's disease (WD) based on imaging indexes, and evaluate the clinical characteristic differences of WD patients at different stages.Methods:Sixty WD patients (40 with cerebral type and 20 with hepatic type) hospitalized in Department of Neurology, First Affiliated Hospital of Sun Yat-sen University from July 2015 to June 2022 and 20 age- and gender-matched normal controls were selected. All subjects accepted susceptibility-weighted imaging (SWI), diffusion tensor imaging (DTI), magnetic resonance spectroscopy (MRS); 7 regions of interest were selected: globus pallidus, caudate nucleus, putamen, thalamus, cerebellum, midbrain and pons; their fractional anisotropy (FA), corrected phase (CP), N-acetylaspartate/creatine (NAA/Cr) values were measured. According to DTI, SWI and MRS results, WD patients were divided into group of metal deposition stage (decreased CP, normal FA and NAA/Cr), group of fiber damage stage (abnormal FA, normal NAA/Cr), and group of neuron necrosis stage (decreased NAA/Cr); the clinical data (modified Young scale scores, Child-Pugh grading of liver function, serum copper content, and urinary copper content) and imaging indexes (FA, CP, and NAA/Cr) among the 3 groups and control group were compared. Results:Among the 60 patients, 19 patients were at metal deposition stage (including 18 with liver type and 1 with brain type), 28 patients at fiber injury stage (including 2 with liver type and 26 with brain type), and 13 patients at neuron necrosis stage (all brain type). (1) Compared with group of metal deposition stage and fiber damage stage, group of neuron necrosis stage had significantly decreased urinary copper content ( P<0.05). The modified Young scale scores in groups of metal deposition stage, fiber injury stage and neuronal necrosis stage increased successively; Child-Pugh grading in group of metal deposition stage was higher than that in groups of fiber injury stage and neuronal necrosis stage. (2) Compared with groups of metal deposition stage and neuron necrosis stage, CP values in the globus pallidus and substantia nigra in group of fiber injury stage group were significantly decreased ( P<0.05). Compared with groups of metal deposition stage and fiber injury stage, the FA value in the putamen and NAA/Cr value in the pallidum, thalamus and caudate nucleus in group of neuron necrosis stage were significantly decreased ( P<0.05). Conclusion:Disease stages of WD patients can be divided by imaging methods; neurological symptoms gradually worsen following progressed WD.