Corpus callosum abnormalities in Wilson's disease.
BACKGROUND
Wilson's disease (WD) with neurological presentation is associated with brain lesions classically localised in globus pallidus, putamen, thalamus, mesencephalon, pons and dentate nucleus. Lesions of corpus callosum (CC) have not been studied in a broad population of patients with WD.


OBJECTIVE
Evaluation of the frequency of CC lesions in patients with neurological symptoms related to WD.


METHODS
The authors included all patients with neurological expression of WD, followed in the French national centre for WD who had a brain MRI between March 2006 and December 2008. The localisation of brain lesions was analysed and the frequency of lesions in CC evaluated. All patients were assessed using the Unified Wilson's Disease Rating Scale. For patients with abnormalities located in CC, a clinical dysconnexion syndrome was investigated.


RESULTS
Among 81 patients (45 men, mean age: 34.8 years, from 12 to 74 years) with neurological expression, 42% had white-matter lesions on fluid-attenuated inversion recovery MRI. 23.4% of patients presented CC lesions, limited to the posterior part (splenium). The severity of disability estimated by Unified Wilson's Disease Rating Scale was correlated with the presence of CC lesions on MRI.


CONCLUSIONS
Abnormalities in CC are not unusual (23.4%). Together with lesions of basal ganglia, CC signal changes should suggest the diagnosis of WD.
Trocello
J M
JM
French National Centre of Wilson’s disease, Lariboisière Hospital, Paris, France. jean-marc.trocello@lrb.aphp.fr
Guichard
J P
JP
Leyendecker
A
A
Pernon
M
M
Chaine
P
P
El Balkhi
S
S
Poupon
J
J
Chappuis
P
P
Woimant
F
F
D000293:Adolescent; D000328:Adult; D000368:Aged; D001479:Basal Ganglia / Q000473:pathology; D002648:Child; D003337:Corpus Callosum / Q000473:pathology*; D005260:Female; D005500:Follow-Up Studies; D006527:Hepatolenticular Degeneration / Q000175:diagnosis*; D006801:Humans; D007089:Image Enhancement; D007091:Image Processing, Computer-Assisted; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009460:Neurologic Examination; D055815:Young Adult