Mid-brain pathology of Wilson's disease: MRI analysis of three cases.

MRI scans were obtained from three patients with Wilson's disease, all of whom showed rigidity and dysarthria; two also showed tremor and dystonia. Two had been treated with D-penicillamine for seven and 14 years, respectively and their neurological abnormalities had improved, but the third patient had not been treated. T2-weighted MRI of the mid-brain in all three revealed the characteristic "face of the giant panda" sign, consisting of high signal intensity in the tegmentum except for red nucleus, preservation of signal intensity of the lateral portion of the pars reticulata of the substantia nigra and hypo-intensity of the superior colliculus. The clinical significance of these MRI abnormalities is discussed.

[1]  M. Ohno,et al.  [MR imaging of the brain in Wilson's disease]. , 1989, Rinsho hoshasen. Clinical radiography.

[2]  S. Fahn,et al.  Study of movement disorders and brain iron by MR. , 1987, AJR. American journal of roentgenology.

[3]  A. Young,et al.  Clinical assessment of 31 patients with Wilson's disease. Correlations with structural changes on magnetic resonance imaging. , 1987, Archives of neurology.

[4]  G. Allan Johnson,et al.  Magnetic Resonance Imaging of Brain Iron , 1986 .

[5]  A. Young,et al.  Wilson disease of the brain: MR imaging. , 1985, Radiology.

[6]  I. Young,et al.  Nuclear Magnetic Resonance (NMR) Imaging in Wilson Disease , 1983, Journal of computer assisted tomography.

[7]  Y. Kuroiwa,et al.  CT manifestation of cerebral white matter lesion in Wilson disease , 1983, Annals of neurology.

[8]  F. J. Williams,et al.  Wilson's disease. An analysis of the cranial computerized tomographic appearances found in 60 patients and the changes in response to treatment with chelating agents. , 1981, Brain : a journal of neurology.

[9]  D. Howse,et al.  Computerized cranial tomography in Wilson disease , 1979, Neurology.

[10]  K. Davis,et al.  Computed tomography in wilson disease: Report of 2 cases , 1979, Annals of neurology.

[11]  P. J. Warren,et al.  The distribution of copper in human brain. , 1960, Brain : a journal of neurology.

[12]  J. Cumings.  The copper and iron content of brain and liver in the normal and in hepato-lenticular degeneration. , 1948, Brain : a journal of neurology.

[13]  S. A. Wilson,et al.  PROGRESSIVE LENTICULAR DEGENERATION: A FAMILIAL NERVOUS DISEASE ASSOCIATED WITH CIRRHOSIS OF THE LIVER , 1912 .

[14]  Zorina La,et al.  The copper and iron content of the blood in healthy persons and in certain blood diseases , 1958 .