Long-term therapy with cytochrome c, flavin mononucleotide and thiamine diphosphate for a patient with Kearns-Sayre syndrome.
Cardiocrome, containing cytochrome c, flavin mononucleotide and thiamine diphosphate, was administered intravenously for 22 months to a patient with Kearns-Sayre syndrome. This combined therapy alleviated the patient's easy fatigability, motor disability, corneal edema and chilblains, but was not effective for his ophthalmoplegia, blepharoptosis or hearing loss. Truncal ataxia, dysphagia and an atrioventricular block appeared even with this therapy. Although the abnormal distribution of cerebral blood flow demonstrated by single photon emission computed tomography was improved, serial cranial magnetic resonance imaging and electrophysiological examination revealed progressive changes. In conclusion, this therapy was favorably effective for impaired skeletal muscle function and corneal edema, but not for ocular movements, central nervous system symptoms or cardiac conduction abnormalities, because irreversible degeneration had probably occurred in these organs.
Department of Child Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders, National Institute of Neuroscience, National Center of Neurology and Psychiatry (NCNP), Kodaira, Tokyo, Japan.
E
E
Nakagawa
S
S
Osari
H
H
Yamanouchi
H
H
Matsuda
Y
Y
Goto
I
I
Nonaka
D003574:Cytochrome c Group / Q000008:administration & dosage*; D004359:Drug Therapy, Combination; D004569:Electroencephalography; D004594:Electrophysiology; D016057:Evoked Potentials, Auditory, Brain Stem; D005486:Flavin Mononucleotide / Q000008:administration & dosage*; D006327:Heart Block / Q000175:diagnosis; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D017237:Mitochondrial Encephalomyopathies / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology; D013835:Thiamine Pyrophosphate / Q000008:administration & dosage*; D013997:Time Factors; D015899:Tomography, Emission-Computed, Single-Photon
D003574:Cytochrome c Group; D005486:Flavin Mononucleotide; D013835:Thiamine Pyrophosphate