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Sonographic detection of basal ganglia lesions in asymptomatic and symptomatic Wilson disease

From the Department of Neurology (Drs. Walter, Benecke, and Dressler), University of Rostock, Germany; Institute of Psychiatry and Neurology (Drs. Krolikowski, Tarnacka, and Czlonkowska), 2nd Department of Neurology, Warsaw; and Medical Academy (Drs. Krolikowski and Czlonkowska), Department of Pharmacology, Warsaw, Poland.

Address correspondence and reprint requests to Dr. Uwe Walter, Department of Neurology, University of Rostock, Gehlsheimer Str. 20, D-18147 Rostock, Germany; e-mail: uwe.walter{at}med.uni-rostock.de

Objective: To investigate whether transcranial brain parenchyma sonography (TCS) detects basal ganglia abnormalities in asymptomatic and symptomatic patients with Wilson disease (WD) and whether findings correlate with disease severity.

Methods: Twenty-one patients with WD with (n = 18) or without (n = 3) neurologic symptoms were investigated. Disease severity was assessed by three independent neurologists using a WD rating scale (WDRS) with the items dysarthria, akinesia, ataxia, tremor, and dystonia; the raters’ median score was used for further analysis. Basal ganglia TCS was performed according to a standardized protocol.

Results: TCS revealed lenticular nucleus (LN) hyperechogenicity in all assessable neurologically symptomatic and in two of the three asymptomatic patients. Size of LN hyperechogenic area correlated with the WDRS score (Spearman correlation, rho = 0.604, p = 0.006), as did the size of thalamus hyperechogenic area (n = 7, rho = 0.891, p = 0.007), the width of third ventricle (n = 21, rho = 0.613, p = 0.003), and the width of lateral ventricles (n = 20, rho = 0.642, p < 0.001). Substantia nigra hyperechogenicity, detected in 10 patients, did not correlate with disease severity. There was no correlation between age at disease onset or disease duration and any TCS finding. Of the 19 patients with LN hyperechogenicity, only 12 showed abnormal LN on MRI.

Conclusions: Transcranial brain parenchyma sonography (TCS) detects lenticular nucleus hyperechogenicity, likely to be caused by copper accumulation, in neurologically symptomatic and asymptomatic Wilson disease (WD). TCS findings correlate with disease severity. TCS appears a promising tool for disease monitoring in WD.

Received December 1, 2004. Accepted in final form February 2, 2005.

This article has been cited by other articles:

				U. Walter, D. Dressler, T. Probst, A. Wolters, M. Abu-Mugheisib, M. Wittstock, and R. Benecke Transcranial Brain Sonography Findings in Discriminating Between Parkinsonism and Idiopathic Parkinson Disease Arch Neurol, November 1, 2007; 64(11): 1635 - 1640. [Abstract] [Full Text] [PDF]