A case of acute hyperammonemic encephalopathy with extensive cortical high-intensity lesions in diffusion weighted imaging

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  • MRI拡散強調画像で広範な大脳皮質病変をみとめた急性高アンモニア血症性脳症の1例

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<p>A 49-year-old woman with hepatic failure owing to alcoholic liver cirrhosis went into a deep coma. Her serum ammonia concentration was elevated at 436 μg/dl, and she had a generalized convulsion. Electroencephalogram and cerebrospinal fluid examination did not suggest encephalitis and epilepsy. Hyperammonemia may be occur because of generalized convulsions; however, it can spontaneously resolve if the convulsions are stopped. If hyperammonemia is the primary cause of generalized convulsion, the serum ammonia concentration will remain high until the cause is eliminated. However, despite stopping the convulsions, her ammonia concentration remained high. Diffusion-weighted brain MRI revealed symmetric high-intensity lesions in the frontal, temporal, and parietal cortices, especially the cingulate and insular cortices of the bilateral hemispheres. These findings were in line with those of previous reports that revealed symmetric cortical lesions, including cingulate and insula cortices that are distinctive in acute hyperammonemic encephalopathy. Therefore, we diagnosed that the coma, generalized convulsions, and abnormal brain MRI findings were caused by acute hyperammonemic encephalopathy.</p>

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  • Rinsho Shinkeigaku

    Rinsho Shinkeigaku 59 (10), 659-661, 2019

    Societas Neurologica Japonica

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