Wilson’s disease: two treatment modalities. Correlations to pretreatment and posttreatment brain MRI

Abstract

From the neurological point of view, there was no difference on the evolution between the group treated exclusively with D-P and the one treated with Zn. Analysis of MRI scans with longer intervals after the beginning of treatment depicted a trend for neuroimaging worsening, without neurological correspondence, among patients treated with Zn. Neuroimaging pattern of evolution was more favorable for the group that received exclusively D-P.

The effect of gender on brain MRI pathology in Wilson’s disease

“…In general, we found less frequent brain MRI abnormalities compared with previous reports (King et al 1996; Da Costa et al 2009; Prashanth et al 2005; Magalhaes et al 1994; Saatci et al 1997; Prayer et al 1990; Kozic et al 2003), which may be due to differences in the methods of patient selection, the examination performed at diagnosis, patients with all of the clinical manifestations (hepatic neuropsychiatric and presymptomatic), and the large number of cases. Until now, most studies have presented data obtained mainly from neurological patients (Machado et al 2006; King et al 1996; Da Costa et al 2009; Prashanth et al 2005; Magalhaes et al 1994; Saatci et al 1997), with very few patients exhibiting the hepatic form of the disease (Kozic et al 2003).…”

“…Until now, most studies have presented data obtained mainly from neurological patients (Machado et al 2006; King et al 1996; Da Costa et al 2009; Prashanth et al 2005; Magalhaes et al 1994; Saatci et al 1997), with very few patients exhibiting the hepatic form of the disease (Kozic et al 2003). We believe that our data are not deeply biased by our hospital speciality because we had approximately 30 % of patients with hepatic manifestation at diagnosis.…”

“…In previous WD studies, brain MRI atrophic changes have been reported from 82 % (King et al 1996) to 25 % in the hepatic form (Kozic et al 2003). The occurrence of MRI atrophic changes has been proposed to be related to the clinical predominant form, particularly when the disease was untreated and neuropsychiatric symptoms appeared (Da Costa et al 2009). The more frequent occurrence of brain cortical atrophy and cerebellar atrophy in WD men compared to women may be explained by gender-related differences in clinical presentation.…”

“…In WD patients with neurological signs (Machado et al 2006), localisation of brain magnetic resonance imaging (MRI) lesions are well described (King et al 1996; Da Costa et al 2009; Prashanth et al 2005; Magalhaes et al 1994; Saatci et al 1997; Prayer et al 1990; Marsden 1987) but the clinical significance of these findings remains unclear. Published MRI studies of WD have shown a number of abnormalities, including focal atrophy, high intensity lesions and hypointense areas on T2-weighted imaging (King et al 1996; Da Costa et al 2009; Prashanth et al 2005; Magalhaes et al 1994; Saatci et al 1997; Prayer et al 1990). Typical WD brain MRI changes are described as symmetrical T2 hyperintensity or mixed intensity in the putamina (with a hyperintense peripheral putamina rim) and globi pallidi, caudate nuclei, thalami and pons.…”

Regional morphometric abnormalities and clinical relevance in Wilson's disease

“…9 Copper accumulation in the brain can induce changes such as myelin degeneration, gliosis, and profound neuronal loss. 10,11 Among all brain structures (including the cerebellum), the gray matter in the basal ganglia [11][12][13][14][15] is affected the most, followed by the brainstem (red nucleus and substantia nigra [SN]) 16,17 and the cortex (especially the frontal lobes and the parietooccipital zones). 14,18 Additionally, emotion research has revealed that the psychiatric impairments of WD, including emotional lability, personality disorders, and mania, are caused by abnormalities in the limbic system 19 and basal ganglia.…”