Hyperammonemia with intermittent loss of consciousness caused by chronic portal-systemic shunt encephalopathy (CPSE) in hemodialysis patient-A case report

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  • Ｃｈｒｏｎｉｃ　ｐｏｒｔａｌ‐ｓｙｓｔｅｍｉｃ　ｓｈｕｎｔ　ｅｎｃｅｐｈａｌｏｐａｔｈｙ（ＣＰＳＥ）による高アンモニア血症のために意識障害を反復した１例
  • 症例報告 Chronic portal-systemic shunt encephalopathy(CPSE)による高アンモニア血症のために意識障害を反復した1例
  • ショウレイ ホウコク Chronic portal systemic shunt encephalopathy CPSE ニ ヨル コウアンモニア ケツショウ ノ タメニ イシキ ショウガイ オ ハンプクシタ 1レイ

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Abstract

A 76-year-old female on chronic hemodialysis developed portal-systemic shunt encephalopathy. She has undergone maintenance hemodialysis for diabetic nephropathy since November 1997. She previously underwent right nephrectomy for renal cell carcinoma and partial gastrectomy for gastric cancer. In May 2005, she began to experience intermittent loss of consciousness. Computed tomography (CT) scan of the brain did not show any remarkable abnormality, thus, the episodes had been considered transient ischemic attacks. On June 2, 2005, she was admitted to a hospital for the same symptoms and her conscious level decreased during hemodialysis treatment. Flapping tremor and an ammonia smell suggesting hyperammnonemic coma were noted ; therefore, AMINOLEBAN®, a solution containing branched-chain amino acids, was administered by drip infusion and she regained consciousness. The serum ammonia level was increased to 286 μg/dL. The results of laboratory examinations did not indicate any evidence of liver damage. Abdominal enhanced CT and angiography showed a gastrorenal shunt. She was diagnosed as having encephalopathy associated with hyperammnonemia due to chronic portal-systemic shunt. Balloon-occluded retrograde transvenous obliteration (B-RTO) was performed. Despite incomplete occlusion of the gastrorenal shunt, the serum ammonia level was reduced to about 130 μg/dL, and there has not been any recurrence of encephalopathy during 2-year follow-up.

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