書誌事項
- タイトル別名
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- A case of ornithine transcarbamylase deficiency associated with acute left hemiplegia.
抄録
A fifteen-month-old girl was admitted with recurrent vomiting, irritability and acute left hemiplegia. She then became somnolent, and showed hyperammonemia and liver dysfunction. Cranial CT scanning revealed widespread low density area of the right cerebral hemisphere and the right-to-left shift of the midline with brain edema. Reye syndrome was suspected by microvesicular fat-droplet deposition of the liver biopsy speci-men. But the results of her serum amino acid analysis and protein-loading test showed that she had ornithine transcarbamylase (OTC) deficiency. The etiology of hemiplegia remained to be proved in spite of cerebral angiography. Usually neurological symptoms of OTC defeciency are non-specific. There has been no such a report that the patient with OTC deficiency developed acute hemiplegia and showed widespread low density area of the cerebral hemisphere as in our case. This paper emphasizes the variability of neurological features of OTC deficiency.
収録刊行物
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- 脳と発達
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脳と発達 20 (3), 232-236, 1988
THE JAPANESE SOCIETY OF CHILD NEUROLOGY