Aims: This study evaluates brain MRI changes and its clinical associations in children with Wilson disease.

Method: Sixty five consecutive cases of the hospitalized pediatric patients of Wilson disease were reviewed. 26 cases are neurologic type, and 39 cases are non-neurologic type according to the proposal by Ferenci. Brain MR examinations were performed in 43 children, which were retrospectively analyzed.

Result: Among the neurologic type, 23 cases of brain MRI were reviewed. All were abnormal, except for one case with psychological symptoms. Eight of 20 non-neurologic type who underwent MRI had abnormal signals. Basal ganglia involvement is the most both in neurologic and non-neurologic type [20(91%) vs 6(75%)]. Ventricular enlargement [7(31.8%)vs 2(25%)]、cerebral atrophy [4(18.2%)vs 2(25%)] and white matter involved [4(18.2%)vs 2(25%)] are also common. Thalamus [9(41%)] and brain stem [6(27.3%)]are only showed in neurologic type. The earliest ages that present abnormal signals of basal ganglia、ventricular enlargement、brain atrophy、white matter are 8 years old, while thalamus and brain stem involvements are showed above 12 years.

Conclusion: Many of non-neurologic Wilson disease can present abnormal MRI signals in young children. Thalamus and brain stem involvements are only seen in neurologic type and in their adolescence.