Non Wilsonian Hepatocerebral Degeneration

Case Presentation 47 y/o WF with hx of hepatitis C and alcoholic cirrhosis Brought by the family to ER in Nov 2003 because of: Confusion Sudden loss of balance Abnormal movements of the upper extremities Admitted at another hospital 1 month before with hepatic encephalopahty

Past Medical History Hepatitis C, unable to finish treatment because of side effects Recurrent upper GI bleeding secondary to small bowel angiodisplasias TIPS placed in May 2003 Few months after TIPS, she started with hepatic encephalopathy that became more frequent and severe

Physical Examination Awake, but disoriented Cardiopulmonary: normal Abdomen: soft, nontender, no ascites, no HSM Neurological: Choreoatetoid movements of the upper extremities Ataxia Normal strenght and reflexes No clonus, no babinski

Her encephalopathy improved with medical treatment, however her involuntary movements of the upper extremities continued MRI on T1 showed hyperintense signal on bilateral globus pallidus She had normal copper in urine, normal ceruloplasmin and no evidence of Kayser-Fleischer rings

Evolution She was transplanted in December 2003 because of recurrent upper GI bleeding and abnormal movements OLT complicated by primary non-function After second transplantation her clinical status improved without anymore GI bleeding or CNS manifestations

Eight months postOLT (July 2004) patient presents again with complains of confusion associated to high levels of ammonia. Her MRI lesions improved

Eleven months postOLT (November 2004) she presents again with abnormal involuntary movements of the trunk and upper extremities (choreoatetoid) Her MRI findings are similar to the previous time with hyperintense signal on T1 in the basal ganglia and cerebellum

Through work up including doppler, MRI and angiography for possible shunts: negative Liver biopsy done to look for inborn errors of metabolism like OTC (ornithine transcarbamylase deficiency) were ruled out