Case report Wilson’s disease: ‘face of giant panda’ and ‘trident’ signs together
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چکیده
Wilson’s disease is an inborn error of metabolism characterized by inability to excrete copper into the bile, with excessive deposition of copper into the eyes, liver and brain. Lentiform nuclei are involved most commonly, but involvement of thalamus, midbrain and pons results in certain characteristic radiological signs on neuroimaging. Atrophy of cerebral and cerebellar cortex is also common yet under-recognized. Identification of these signs helps in the diagnosis in appropriate clinical setting.
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Wilson's disease: ‘face of giant panda’ and ‘trident’ signs together
Wilson's disease is an inborn error of metabolism characterized by inability to excrete copper into the bile, with excessive deposition of copper into the eyes, liver and brain. Lentiform nuclei are involved most commonly, but involvement of thalamus, midbrain and pons results in certain characteristic radiological signs on neuroimaging. Atrophy of cerebral and cerebellar cortex is also common ...
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*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 accumulati...
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