title: Does brain degeneration in Wilson disease involve not only copper but also iron accumulation?
abstract: OBJECTIVE
  Wilson disease (WD) is an autosomal recessive inherited disorder of hepatic copper metabolism. Clinical manifestations of WD include neurologic, hepatic and psychiatric symptoms. Most WD patients with the neuropsychiatric form, and some with the hepatic and presymptomatic forms have both hypointense and hyperintense lesions in basal ganglia on T2-weighted magnetic resonance imaging (MRI), which can be iron and copper accumulation. It has been established that T2* and susceptibility-weighted imaging (SWI) are highly sensitive in demonstrating brain iron accumulation, showing decreased signal intensity. Hypointense globus pallidus (GP) signal has been described on T2-, T2*-weighted images and on SWI as typical MRI lesion for patients with neurodegeneration with brain iron accumulation (NBIA). We investigated whether WD patients have MRI changes suggesting iron accumulation using T2*-weighted and VEN_BOLD SWI imaging protocols.
  
  
  METHODS
  Standard MRI with additional sequences (T2*-weighted and VEN_BOLD SWI) was performed in consecutively admitted, clinically stable, and treated patients.
  
  
  RESULTS
  Twenty-eight patients entered the study. Hypointensity in the GP was observed on T2*-weighted images in 10 pa-tients. Using the VEN_BOLD SWI technique, we found hypointense signal in GP in 20 patients.
  
  
  CONCLUSIONS
  MRI data suggest not only copper but also iron accumulation in GP in WD patients.
authors.affiliation: Prof. Anna Członkowska, II Klinika Neurologii, Instytut Psychiatrii i Neurologii, ul. Sobieskiego 9, 02-957 Warszawa, tel.: +48 22 458 26 66, e-mail: czlonkow@ipin.edu.pl.
authors.forename: Marta
authors.initials: M
authors.lastname: Skowrońska
authors.forename: Tomasz
authors.initials: T
authors.lastname: Litwin
authors.forename: Karolina
authors.initials: K
authors.lastname: Dzieżyc
authors.forename: Agata
authors.initials: A
authors.lastname: Wierzchowska
authors.forename: Anna
authors.initials: A
authors.lastname: Członkowska
mesh_terms: D000328:Adult; D001479:Basal Ganglia / Q000737:chemistry* / Q000473:pathology; D001921:Brain / Q000473:pathology*; D001923:Brain Chemistry; D003300:Copper / Q000032:analysis*; D005260:Female; D005917:Globus Pallidus / Q000737:chemistry* / Q000473:pathology; D006527:Hepatolenticular Degeneration / Q000175:diagnosis*; D006801:Humans; D007501:Iron / Q000032:analysis*; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D055815:Young Adult
chemical_list: D003300:Copper; D007501:Iron