“The Perils Of Excess”: Wilson’s Disease ----A Copper Connection

Wilson’s disease aka progressive hepatolenticular degeneration is an unwonted genetic disorder of copper metabolism. Though its pathogenic inception lies within the hepatobiliary system, the brunt of relentless copper accretion is borne across a multisystemic terrain. The complex symptomatology of this syndrome mandates a high index of suspicion as left undiagnosed it has a tenacious progression with resultant severe disability & death. An accurate & timely diagnosis allows initiation of optimal treatment regimens to prevent or revert its protean manifestations. MRIbased neuroimaging serves to elaborate upon the anatomical, biochemical and pathological correlates of the disease and hence not only facilitates diagnosis and severity assessment but is also instrumental in monitoring therapeutic response. We hereby present the case of a 23 years old female with the neurological, psychiatric, hepatic, and ocular manifestations of Wilson’s disease with relevant radiological correlates. The neuroimaging in this case particularly demonstrates the Face of the giant panda and bisected pons signs in addition to cerebral white matter changes all of which are extremely rarely seen concurrently and hence bequeath exclusivity to our case. In addition, the face of the giant panda sign is an infrequent but pathognomonic feature of Wilson’s disease; and, is also the sole MRI feature that serves to differentiate it from all other early onset extrapyramidal disorders.