A 45 year old Alcoholic comes to OPD who is on abstinence since last 10 years.He suddenly develops dysahria, shuffling gait and intention tremors . What shall be the most likely diagnosis ?
Correct Answer: Acquired hepatocerebral degeneration
Description: Disease Features Wilson's ds -Patient will be present at 10 yr age -KF Ring -But here it is alcoholic so ruled out. Wernicke's disease -Global confusion, -Ophthalmoplegia -Ataxia Hepatocerebral degeneration -Rare complication of cirrhosis -Indicates a decompensation in of cirrhosis - Development of Poal HTN - develops poosystemic collateral circulation- | Ammonia (NH3+) in circulation - Neurological c/f. Halloverden spatz disease/NBIA -Degeneration due to iron deposition -Accumulation of iron in putamen * Presentation is in childhood (10-15 yr. age) * It is d/d of Wilson's disease -MRI =iron deposition in putamen given eye of tiger appearance.
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