1. Non Wilsonian Hepatocerebral Degeneration

2. 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

3. 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

4. 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

5. 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

8. 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

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

12. 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

13. 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