A 55-year-old woman with history of ascites for 3 years, bradykinesia and tremors of right upper limb for 6 months.
A
B
C
What is your diagnosis?
A. Creutzfeldt-Jakob disease (CJD)
B. Hepatic encephalopathy
C. Fahr disease
D. Wilson disease
?
?
?
The answer is Hepatic Encephalopathy
Hepatic encephalopathy includes a spectrum of neuropsychiatric abnormalities occurring in patients with liver dysfunction.
Patients with hepatic encephalopathy typically have a rise of manganese levels in the brain, with a preferential deposition in the globus pallidi.
The classic MRI findings are bilateral symmetric T1 hyperintensities in the globus pallidi (A, arrows) that are iso- or hyperintense on T2WI and T2 FLAIR (B and C, arrows) and hyperintense on T1WI, are attributed to manganese deposits, and are reversible after liver transplantation.
Other sites of T1 hyperintensities are the substantia nigra and anterior pituitary gland.
中文版：
女性，55歲，腹水3年，運動遲緩、右上肢震顫6個月
診斷為？
A. 克雅氏病
B. 肝性腦病
C. Fahr病
D. Wilson病
診斷：肝性腦病
肝性腦病為肝功能異常患者發生的一系列神經精神疾病。
肝性腦病患者腦內錳的含量升高，且容易沉積於蒼白球。
典型MRI表現：雙側蒼白球對稱性T1WI高信號（A圖箭頭），T2WI及T2 FLAIR呈等或稍高信號（B,C箭頭），該表現歸因於錳的沉積，肝移植後病變可逆轉。
其它部位也可出現T1WI高信號，比如黑質和垂體前葉。
From AJNR Classic Case