title: Treatment of mitochondrial encephalomyopathy with a combination of cytochrome C and vitamins B1 and B2.
abstract: The therapeutic efficacy of a regimen consisting of intravenous injection of Cardiocrome, containing cytochrome c, flavin mononucleotide and thiamine diphosphate for mitochondrial encephalomyopathy (MEM) was examined. This combined therapy was applied to nine patients with MEM, including four with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes. For the standard regimen, Cardiocrome was first injected daily, usually for 4 weeks, and later by means of intermittent injections for maintenance treatment. Clinical improvement was obtained in eight of the patients. Improvement was observed in the muscle symptoms of easy fatigability, motor disability and severity of stroke-like episodes, as well as in various other symptoms such as phosphate, tinnitus, headache, corneal edema, chilblains, thalamic pain, respiratory failure, and nystagmus. This clinical improvement was maintained for more than 1 year by additional intermittent injections. In conclusion, this therapy was fairly effective for the management of patients with MEM.
authors.affiliation: Department of Pediatrics, Osaka University Medical School, Japan.
authors.forename: J
authors.initials: J
authors.lastname: Tanaka
authors.forename: T
authors.initials: T
authors.lastname: Nagai
authors.forename: H
authors.initials: H
authors.lastname: Arai
authors.forename: K
authors.initials: K
authors.lastname: Inui
authors.forename: H
authors.initials: H
authors.lastname: Yamanouchi
authors.forename: Y
authors.initials: Y
authors.lastname: Goto
authors.forename: I
authors.initials: I
authors.lastname: Nonaka
authors.forename: S
authors.initials: S
authors.lastname: Okada
mesh_terms: D000293:Adolescent; D000328:Adult; D002556:Cerebrospinal Fluid Proteins / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D003574:Cytochrome c Group / Q000008:administration & dosage*; D030401:Cytochrome-c Oxidase Deficiency; D045304:Cytochromes c; D004338:Drug Combinations; D005260:Female; D005486:Flavin Mononucleotide / Q000008:administration & dosage*; D006801:Humans; D007625:Kearns-Sayre Syndrome / Q000188:drug therapy; D007773:Lactates / Q000097:blood / Q000134:cerebrospinal fluid; D017241:MELAS Syndrome / Q000188:drug therapy; D017243:MERRF Syndrome / Q000188:drug therapy; D008297:Male; D017237:Mitochondrial Encephalomyopathies / Q000188:drug therapy*; D012256:Riboflavin / Q000008:administration & dosage*; D013831:Thiamine / Q000008:administration & dosage*; D013835:Thiamine Pyrophosphate / Q000008:administration & dosage*
chemical_list: D002556:Cerebrospinal Fluid Proteins; D003574:Cytochrome c Group; D004338:Drug Combinations; D007773:Lactates; C017112:cytochrome C, flavin mononucleotide, thiamine pyrophosphate drug combination; D005486:Flavin Mononucleotide; D045304:Cytochromes c; D013835:Thiamine Pyrophosphate; D012256:Riboflavin; D013831:Thiamine