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Eur J Neurol ; 28(2): 726-728, 2021 02.
Article in English | MEDLINE | ID: mdl-33022802

ABSTRACT

Wilson's disease (WD), also known as hepatolenticular degeneration, is a rare autosomal recessive disorder that results from abnormal ceruloplasmin metabolism, with copper deposition affecting multiple systems. Osmotic demyelination syndrome (ODS) refers to acute demyelination seen in the setting of osmotic changes, typically with the rapid correction of electrolyte disturbance. We present a 29-year-old male patient diagnosed with WD 1 year after the onset of extrapyramidal symptoms. Magnetic resonance imaging performed during hospitalization showed two patterns of pons involvement, which allowed the diagnosis of ODS in addition to WD. Classic imaging findings were observed and illustrate perfectly these two conditions.


Subject(s)
Demyelinating Diseases , Hepatolenticular Degeneration , Adult , Ceruloplasmin/metabolism , Copper/metabolism , Demyelinating Diseases/diagnostic imaging , Hepatolenticular Degeneration/complications , Hepatolenticular Degeneration/diagnostic imaging , Humans , Magnetic Resonance Imaging , Male
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