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Eur J Neurol. 1999 Sep;6(5):613-5.

Adult leigh syndrome: treatment with intravenous soybean oil for acute central respiratory failure.

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Department of Neurology, Amagasaki Prefectural Hospital, Hyogo, Japan.


This study reports a 38-year-old woman with adult Leigh syndrome associated with partial deficiency of the pyruvate dehydrogenase complex. The patient had intermittent diplopia, loss of vision, dystonia, central respiratory failure and unconsciousness with lactic acidosis. Treatment with an intravenous ketogenic emulsion resulted in rapid clinical and biochemical improvement. In patients with acute respiratory failure under these circumstances, intravenous ketogenic emulsion therapy is worth consideration.

[Indexed for MEDLINE]

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