title: Impaired NADH-CoQ reductase activity in a child with moyamoya syndrome.
abstract: A 33-month-old boy with recurrent stroke-like episodes had angiographic features characteristic of moyamoya syndrome. Mitochondrial encephalomyopathy was suspected because of lactic acidosis and ptosis. Studies of oxidative metabolism on isolated skeletal muscle mitochondria revealed impairment of NADH-coenzyme Q reductase activity. Mitochondrial metabolic disorders may cause moyamoya syndrome when other known associated factors are absent.
authors.affiliation: Department of Neurology, St. Louis University Medical Center, MO 63110.
authors.forename: S
authors.initials: S
authors.lastname: Kotagal
authors.forename: P L
authors.initials: PL
authors.lastname: Peterson
authors.forename: M E
authors.initials: ME
authors.lastname: Martens
authors.forename: C P
authors.initials: CP
authors.lastname: Lee
authors.forename: M
authors.initials: M
authors.lastname: Nigro
authors.forename: C R
authors.initials: CR
authors.lastname: Archer
mesh_terms: D001157:Arterial Occlusive Diseases / Q000201:enzymology*; D002648:Child; D006801:Humans; D008297:Male; D008931:Mitochondria, Muscle / Q000201:enzymology* / Q000473:pathology; D009072:Moyamoya Disease / Q000201:enzymology* / Q000503:physiopathology; D016660:NAD(P)H Dehydrogenase (Quinone); D011808:Quinone Reductases / Q000378:metabolism*
chemical_list: D016660:NAD(P)H Dehydrogenase (Quinone); D011808:Quinone Reductases