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 »  Introduction
 »  Case report
 »  Discussion
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Year : 2000  |  Volume : 48  |  Issue : 4  |  Page : 395-6

'Face of the giant panda' sign in Wilson's disease : revisited.


Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, 695011, India.

Correspondence Address:
Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, 695011, India.

  »  Abstract

We report a patient, with Wilson's disease, who showed the characteristic radiological sign known as 'Face of the giant panda sign' on magnetic resonance imaging (MRI) of the brain.

How to cite this article:
Kuruvilla A, Joseph S. 'Face of the giant panda' sign in Wilson's disease : revisited. Neurol India 2000;48:395


How to cite this URL:
Kuruvilla A, Joseph S. 'Face of the giant panda' sign in Wilson's disease : revisited. Neurol India [serial online] 2000 [cited 2018 Oct 22];48:395. Available from: http://www.neurologyindia.com/text.asp?2000/48/4/395/1490




   »   Introduction Top

Magnetic resonance imaging (MRI) is an efficient method for documenting involvement of the central nervous system in Wilson's disease, thus allowing better anatomical and clinical correlations.[1] The first description of the characteristic 'face of the giant panda' sign on MRI in Wilson's disease was made by Hitoshi et al[2] in 1991. This interesting MRI appearance is a very rare entity and to the best of our knowledge, this is the second reported case in literature and the first such report from India.


   »   Case report Top

A 24 year old lady presented with tremor of upper extremities since 16 years of age. She had past history of fulminant hepatitis, at age 9, from which she had recovered. She was diagnosed as a case of Wilson's disease and was started on D-penicillamine at 17 years of age. She discontinued D-penicillamine after three years of treatment, while she was pregnant. After the child-birth, D-penicillamine was restarted due to increasing tremor. However, she had been noncompliant with this medication. Neurological examination revealed scanning dysarthria and 'wing beating' tremor of upper extremities with distal action tremor. An ophthalmological examination with a slitlamp showed presence of Kayser-Fleischer (KF) ring bilaterally. Serum ceruloplasmin was too low (<25 mg/dl) to be estimated (normal range : 62-140 md/dl), serum copper was 24 µ gm/dl (N : 75-160 µ gm), urinary copper was 30 µ g/day for an ouput of 1500 ml/day. A T2-weighted MRI scan of the brain in axial plane revealed [Figure. 1] extensive hyperintensity of the midbrain with relative sparing of red nucleus, superior colliculus and part of the pars reticulata of substantia nigra with hypointensity of the aqueduct, giving a 'face of the giant panda' appearance. A diagnosis of Wilson's disease was confirmed and the patient was started on D-penicillamine orally. She showed significant improvement in tremor on follow up.


   »   Discussion Top

MRI provides a more elaborate anatomical information than computerised tomography (CT) of brain on the structure of basal ganglia and brain stem. In addition, it provides biochemical information on heavy metal distribution in the brain tissue and hence, is a clearly useful method for evaluating heavy metal storage disease.[2] The original description of the 'face of the giant panda' sign by Hitoshi et al[2] consisted 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 hypointensity of the superior colliculus. Our patient also has a similar picture in the MRI. The exact pathogenesis of the marked hypointensity of the corpus striatum and superior colliculus in the T2weighted MRI is unknown, but it is postulated that the paramagnetic effects of the deposition of heavy metals, such as iron and copper, may be responsible.[2],[3] The content of iron is much greater than that of copper in cases of Wilson's disease and the iron is assumed to play a more important role than copper in reducing the signal intensity in the T2weighted MRI.[2] Although it is difficult to correlate between MRI findings and clinical neurological symptoms,[2] it will be meaningful to do follow up imaging on these patients after treatment. This will also help to see whether the duration of disease process contributes to the MRI changes or that treatment with D-penicillamine may be responsible for them.

 

  »   References Top

1.Magalhaes AC, Caramelli P, Menezes JR et al : Wilson's disease : MRI with clinical correlation. Neuroradiology 1994; 36 (2) : 97-100.   Back to cited text no. 1    
2.Hitoshi S, Iwata M, Yoshikawa K : Mid-brain pathology of Wilson's disease : MRI analysis of three cases. J Neurol Neurosurg Psychiatry1991; 54 (7) : 624-626.   Back to cited text no. 2    
3.Rutledge JN, Hilal SK, Silver AJ et al : Study of movement disorders and brain iron by MR. Am J Roentgenol 1987; 149 : 365-379.   Back to cited text no. 3    

 

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