The Study Of Susceptibility-weighted Imaging In The Brain Of Wilson’s Disease

Background Hepatolenticular degeneration was first described by Kinnier Wilson,which also called Wilson’s disease (WD). WD is an autosomal recessive inheritedmetabolism disorder. The copper metabolism disorder of WD has been confirmed bymany clinical pathological studies, and becomes one of diagnostic criteria of WD. It isthe unique hereditary diseases of nervous system which can be controlled by drug.Penicillamine is the first choice. But some patients’ condition were worse after treatedby penicillamine, so other mineral accumulation except copper in WD were suspected.As we known, copper and iron metabolism are closely related. The further clinicalstudies found that iron metabolism is disorder in WD patients. Susceptibility-weightedimaging (SWI), a new MRI technique, which contrast-based on tissue susceptibilitydifferences, has been shown to measure brain iron deposition.Objective To evaluate the value of SWI in the brain of WD, and to investigate therelationship between the phase values of WD group and serum copper, ceruloplasminand copper oxidase.Methods Twenty patients with WD and20normal controls underwent conventionalMRI and SWI. The phase values were measured on corrected phase images byhand-drawn region of interest, including caudate nucleus, globus pallidus, putamen,thalamus, substantia nigra, and red nucleus. Two-sample t-test was used for comparisonbetween WD groups and normal controls. Spearman rank correlation was used to assessthe correlation between phase values in each deep-gray nuclei of WD and serum copper, ceruloplasmin, copper oxidase.Results The phase values of bilateral caudate nucleus, globus pallidus, putamen,thalamus, substantia nigra, and red nucleus were significantly lower in WD patients thanthose in control group(P<0.05), and bilateral putamen were most significantly. Thephase values of WD group were not significantly related with serum copper,ceruloplasmin and copper oxidase.Conclusion There are paramagnetic mineralization deposition in gray nuclei of WDpatients. Phase value is an effective method to evaluate paramagnetic mineralizationaccumulated in WD．...