Osmotic demyelination syndrome diagnosed radiologically during Wilson's disease investigation.
Wilson’s disease
extrapyramidal syndrome
magnetic resonance imaging
metabolic disorder
osmotic demyelination syndrome
Journal
European journal of neurology
ISSN: 1468-1331
Titre abrégé: Eur J Neurol
Pays: England
ID NLM: 9506311
Informations de publication
Date de publication:
02 2021
02 2021
Historique:
received:
12
06
2020
accepted:
30
09
2020
pubmed:
7
10
2020
medline:
13
8
2021
entrez:
6
10
2020
Statut:
ppublish
Résumé
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.
Substances chimiques
Copper
789U1901C5
Ceruloplasmin
EC 1.16.3.1
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
726-728Informations de copyright
© 2020 European Academy of Neurology.
Références
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Zhong W, Huang Z, Tang X. A study of brain MRI characteristics and clinical features in 76 cases of Wilson's disease. J Clin Neurosci 2019; 59: 167-174.
Oliveira AM, Paulino MV, Vieira APF, et al. Imaging patterns of toxic and metabolic brain disorders. Radiographics 2019; 39: 1672-1695.