Association of Primary Biliary Cirrhosis with Opsoclonus Myoclonus Syndrome

Primary biliary cirrhosis is an autoimmune disorder affecting exclusively liver. There has been association of this autoimmune disease with various other neurological counterparts, but Opsoclonus-Myoclonus Syndrome in conjunction with primary biliary cirrhosis is unreported yet. Opsoclonus-Myoclonus Syndrome is a disorder of eye movement with an ill-defined etiology. It may be idiopathic or present as a paraneoplastic manifestation with a low response to therapeutic modalities. We present a case of primary biliary cirrhosis that developed Opsoclonus-Myoclonus Syndrome during an exaggeration of disease process which responded well to intravenous corticosteroids. Knowledge of association of Primary biliary cirrhosis with Opsoclonus-Myoclonus Syndrome and its good response to treatment in this setting will help in reducing morbidity in similar situations. Key Words: Primary biliary cirrhosis; Opsoclonus-Myoclonus Syndrome DOI: http://dx.doi.org/10.3126/ajms.v2i2.3536 Asian Journal of Medical Sciences 2 (2011) 110-112