Face of giant panda sign in Wilson's disease.

*Senior Resident, Department of Neurology, **Assistant Professor, ***Associate Professor Institute of Human Behaviour And Allied Science (IHBAS), Delhi 110095 Received: 07.05.2012; Accepted: 23.08.2012 W l s o n d i s e a s e i s a n i n b o r n c o p p e r m e t a b o l i s m d i s o r d e r with autosomal recessive pattern of inheritance caused by ATP7B mutation leading to abnormal accumulation of copper in various tissues, particularly in the liver and the brain. Presence of magnetic resonance imaging (MRI) a b n o r m a l i t i e s o c c u r i n v i r t u a l l y 100% of patients with neurological dysfunctional) among the various MRI findings face of giant panda sign, face of miniature panda sign and bright claustrum signs are characteristics. We describe a 16 year old boy presented with limb dystonia, tremor, and cognitive impairment with positive family history of willson’s disease. Besides the other diagnostics marker such as serum copper, ceruloplasmin.and urinary copper he had characteristics MRI findings (Figures 1 A, B and C). Figure 1A showing face of giant panda sign i.e high signal intensity of T2W image in the tegmentum sparing the red nucleus(eyes, black thin arrow), presevation of signal intensity of the pars reticulate of substantia nigra (ears, white arrow), low signal intensity of superior colliculous (chin, black thick arrow). Figure 1B showed thin rim of hyperintensity in T2W image in claustrum Face of Giant Panda Sign in Wilson’s Disease