Structural Changes of the Brain in Patients with Hepatocerebral Degeneration

I.K. Voloshin-Gaponov

Abstract


The article deals with severe hereditary disease with imbalance between an intake and excretion of copper. This disease occurs more often than it is diagnosed. Establishing diagnosis and, therefore, an effective treatment is often delayed for a few years.
Based on MRI examination of 32 patients with hepatocerebral degeneration (HCD) and МR-spectroscopy of 26 patients with HCD, it was showed that structural abnormalities in the brain of patients with HCD had diffuse character with the primary bilateral defeat of grey substance of basal ganglia and brainstem. The white substance of the brain is involved in a less degree. The phase of neurological manifestations of the disease is always accompanied by atrophic processes in the brain. MRI and МR-spectroscopy enable to improve the accuracy of making diagnosis of HCD and to determine the efficiency of therapy, which is carried out using copper chelates.


Keywords


hepatocerebral degeneration; MRI; МR-spectroscopy; copper metabolism; diagnosis

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DOI: https://doi.org/10.22141/2224-0713.2.56.2013.85796

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