title: Adult leigh syndrome: treatment with intravenous soybean oil for acute central respiratory failure.
abstract: 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.
authors.affiliation: Department of Neurology, Amagasaki Prefectural Hospital, Hyogo, Japan.
authors.forename: R
authors.initials: R
authors.lastname: Kumagai
authors.forename: K
authors.initials: K
authors.lastname: Ichikawa
authors.forename: T
authors.initials: T
authors.lastname: Yasui
authors.forename: Y
authors.initials: Y
authors.lastname: Kageyama
authors.forename: S
authors.initials: S
authors.lastname: Miyabayashi
mesh_terms: D000208:Acute Disease; D000328:Adult; D004032:Diet; D005217:Fat Emulsions, Intravenous; D005260:Female; D006801:Humans; D007275:Injections, Intravenous; D007659:Ketones / Q000097:blood; D019344:Lactic Acid / Q000097:blood; D007888:Leigh Disease / Q000188:drug therapy*; D009460:Neurologic Examination; D015325:Pyruvate Dehydrogenase Complex Deficiency Disease / Q000503:physiopathology; D012131:Respiratory Insufficiency / Q000188:drug therapy* / Q000209:etiology; D013024:Soybean Oil / Q000008:administration & dosage / Q000627:therapeutic use*
chemical_list: D005217:Fat Emulsions, Intravenous; D007659:Ketones; D019344:Lactic Acid; D013024:Soybean Oil