Wilson's disease tremor is associated with magnetic resonance imaging lesions in basal ganglia structures.
Wilson's disease (WD) is an inherited disorder of copper metabolism yielding marked motor deficits, including a severely disabling tremor. As a structural correlate of the disease, a variety of cerebral abnormalities has been revealed. However, the relationship between motor deficits and cerebral lesions has remained largely unknown. Here, we investigated correlation between WD tremor and cerebral magnetic resonance imaging (MRI) findings. Cerebral MRI abnormalities in 6 symptomatic WD patients were compared to findings in 6 asymptomatic WD patients and 10 healthy controls. All patients were treated with long-term copper chelating therapy. Motor symptoms including tremor were determined by Unified Parkinson's Disease Rating Scale Part III (UPDRS-III). MRI findings in symptomatic WD patients revealed significant symmetric T2*-weighted hypointense signal alterations of globus pallidus, head of the caudate nucleus, and substantia nigra. In contrast, MRI of asymptomatic WD patients did not differ from healthy controls. Correlation analysis revealed a significant positive correlation between MRI basal ganglia lesions and UPDRS action tremor score. Our results demonstrate for the first time that Wilson's disease tremor is associated with lesions of the globus pallidus, the head of the caudate nucleus, and the substantia nigra.
Südmeyer
Martin
M
Department of Neurology, Heinrich-Heine University, Düsseldorf, Germany.
Saleh
Andreas
A
Wojtecki
Lars
L
Cohnen
Mathias
M
Gross
Joachim
J
Ploner
Markus
M
Hefter
Harald
H
Timmermann
Lars
L
Schnitzler
Alfons
A
D000328:Adult; D001479:Basal Ganglia / Q000473:pathology*; D002570:Ceruloplasmin / Q000378:metabolism; D003300:Copper / Q000097:blood / Q000652:urine; D005260:Female; D006527:Hepatolenticular Degeneration / Q000097:blood / Q000473:pathology* / Q000652:urine; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009460:Neurologic Examination / Q000379:methods; D012720:Severity of Illness Index; D013223:Statistics as Topic
D003300:Copper; D002570:Ceruloplasmin