MR spectroscopy in monitoring the treatment of Wilson's disease patients

Abstract

The aim of this study was to determine the effectiveness of brain proton magnetic resonance spectroscopy ((1)H-MRS) for monitoring therapy in Wilson's disease (WD) patients. Voxels were located in the globus pallidus (right, left). We followed 17 newly diagnosed WD cases for 1-year period. During this observation period, 6 neurological and 9 hepatic patients improved, while 2 neurological patients deteriorated. The pretreatment (1)H-MRS analysis showed a statistically significant lower level of mI/Cr, NAA/Cr, and higher Lip/Cr in all WD patients with improvement compared with controls. In patients with hepatic signs, a statistically significant increase of mI/Cr and Glx/Cr was observed in the second (1 year posttreatment) (1)H-MRS. In patients with neurological improvement after treatment in the follow-up (1)H-MRS, a statistically significant increase of NAA/Cr was noted. During neurological deterioration, a decrease of Glx/Cr and NAA/Cr was seen, in contrast to another neurologically impaired patient with liver failure exacerbation, where a decrease of mI/Cr and increase of Glx/Cr was observed. The alternations of NAA/Cr ratio in neurologically impaired patients and mI/Cr and Glx/Cr in patients with liver failure could be a sensitive marker of the clinical recovery and deterioration in those WD patients. (1)H-MRS is a technique that can be used for accurate monitoring of treatment efficacy in WD patients.

Improvement of Diffusion Tensor Imaging (DTI) Parameters with Decoppering Treatment in Wilson’s Disease

“…Reversible mitochondrial dysfunction is supported by the previous MRS study where increase in NAA/Cr ratio was noted following initiation of treatment. Presence of neuroinflammation has been reported to be present in the rat model of copper toxicity (Tarnacka et al 2008). Demyelination and inflammatory changes have also been noted in the previous pathology study of Meenakshi-Sundaram et al (2002).…”

“…In a quantitative MRS study, authors reported significant improvement in the NAA/Cr ratio in patients who showed neurological improvement following treatment with decoppering therapy. Neurological deterioration was mirrored by fall in Glx/Cr and NAA/Cr ratios, while hepatic deterioration caused decreased mI/Cr and increased Glx/Cr ratios (Tarnacka et al 2008). In our study, none of the patients reported significant neurological deterioration during the period of serial evaluation.…”

“…However, in previous studies, effects of treatment on DTI measurements have not been evaluated. Effects of decoppering treatment on MRI have been studied using conventional MRI and MR spectroscopy (Sinha et al 2007a, b;Kim et al 2006;Tarnacka et al 2008). Conventional MRI findings show improvement with prolonged treatment but are more often subjective.…”

Hepatobiliary Quiz (Answers)—15 (2015)

“…However, at very early stages of both disorders the picture could quite similar. 53 MRS changes observed in HE are reversible after liver transplantation or pharmacological treatment. 35,54,55 8.…”

“…These can also be seen on a computed tomography (CT) scan. 53 MRS can be used to analyze the concentrations of various metabolites. In WD there is a decrease in N-acetyl-aspartate (NAA), which is a characteristic marker of neuron damage and depletion.…”