Wilson disease, also known as hepatolenticular degeneration, is a rare autosomal recessive disorder of copper metabolism affecting multiple systems. Wilson s disease in brain m agnetic resonance spectroscopy 75 cerebellum and cerebral cortex can be seen hoogenraad, 1997. Mrs is currently used to investigate a number of diseases in the human body. The technique of magnetic resonance spectroscopy usually shortened to mr. Mri is much more sensitive method for revealing abnormalities in wd hoogenraad, 1997 sinha et al.
Mri of the brain and liver using t2 relaxation time measurements and proton spectroscopy 1hmrs of the brain was performed in four siblings with wilsons dis ease one with clinical disease and three asymptomatic as well as age and sexmatched control subjects. Identification of diffuse and focal brain lesions by clinical magnetic. To our knowledge, this is the first time that mrs was. Wilsons dis ease or progressive hepatolenticular degeneration is an autosomal recessive disorder of copper metabolism. Handbook of magnetic resonance spectroscopy in vivo. Magnetic resonance imaging and proton mr spectroscopy in wilsons disease. In addition to the three books and the paper mentioned above, it is. Grossman and yousem said if you need this to help you, go back to page 1. Neurologic wilson disease is an inherited disease characterized by a copper metabolic disorder that causes damage to many organs, especially the brain. Wilson s disease in brain magnetic resonance spectroscopy, magnetic resonance spectroscopy, donghyun kim, intechopen, doi.
Therefore, we investigated the correlation of brain abnormalities in patients with neurologic wilson disease with their. Brain magnetic resonance spectroscopy mrs as a diagnostic tool for detecting early neurological changes in children with wilsons disease. On t1weighted images, generalized brain atrophy is seen in about 75% of cases, and. The gene that causes wilsons dis ease, located at chromosome band q14. In demyelinating diseases, we had reliably increased myoinositol mi peaks, and the metabolic disease group showed raised glutamate glx peak. Brain magnetic resonance spectroscopy mrs as a diagnostic tool. Mr imaging of the brain in neurologic wilson disease. Wilson disease wd is rare but one of the few metabolic disorders that can possibly benefit from effective available treatments. In this book, topics of mrs both relevant to the clinic and also those that are. Mr spectroscopy in multiple sclerosis a new piece of the puzzle. Magnetic resonance spectroscopy mrs of the brain can. In vivo magnetic resonance spectroscopy news newspapers books scholar jstor may 2010 learn how and when to remove this template message. Wilson s disease in brain magnetic resonance spectroscopy. Typical psychiatric symptoms occur in almost 65% wd patients2931 and include personality disorders, mood disorders, cognitive impairment, psychosis, and less frequently other psychiatric alterations anorexia, catatonia, substance abuse and others.
In vivo magnetic resonance spectroscopy mrs is a specialized technique associated with. A mutation of the gene associated with wilsons dis ease results in deficient biliary excretion of copper, leading to excessive copper. The literature regarding proton mr spectroscopy mrs in wd is scarce and controversial. Mrs theory, practice and applications emagres books 1st edition. We reported the brain proton magnetic resonance spectroscopy mrs findings in 27 wilson s disease wd patients treated more than 6 years in neurological nwd and hepatic hwd subgroups. Magnetic resonance imaging and proton mr spectroscopy in. Few studies report the relationships between these neurologic symptoms and mr imaging of the brain. Wilsons disease in brain magnetic resonance spectroscopy. Mr spectroscopy evaluation of white matter signal abnormalities of. This is perhaps a little harsh, however, it is fair to say that mrs. Canavans disease, krabbe disease globoid cell leukodystrophy, leighs disease. Clinical application of liver mr imaging in wilsons disease. The purpose of this study was to determine the brain metabolic changes due to wd by using mrs. The technique of magnetic resonance spectroscopy usually shortened to mr spectroscopy or mrs allows tissue to be interrogated for the presence and concentration of various metabolites.
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