title: Recurrent pain attacks in a 3-year-old patient with myoclonus epilepsy associated with ragged-red fibers (MERRF): a single-photon emission computed tomographic (SPECT) and electrophysiological study.
abstract: We reported a 3-year-old girl with myoclonus epilepsy associated with ragged-red fibers (MERRF) who was afflicted with recurrent pain attacks and allodynia on the right side of the body. Although magnetic resonance imaging showed normal intensity in the thalamus, single-photon emission computed tomography (SPECT) revealed hypoperfusion in the thalamus. Somatosensory evoked potentials showed delayed early cortical responses, particularly on right median nerve stimulation. The parenteral administration of cytochrome c with flavin mononucleotide and thiamine diphosphate abolished the intolerable pain. This clinical improvement was objectively supported by the results of SPECT and neurophysiological findings. These observations suggested that a dysfunction of the thalamus was responsible for her pain and that cytochrome c therapy was of benefit for this symptom.
authors.affiliation: Department of Child Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders, Tokyo, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Tanaka
authors.forename: S
authors.initials: S
authors.lastname: Osari
authors.forename: M
authors.initials: M
authors.lastname: Ozawa
authors.forename: H
authors.initials: H
authors.lastname: Yamanouchi
authors.forename: Y
authors.initials: Y
authors.lastname: Goto
authors.forename: H
authors.initials: H
authors.lastname: Matsuda
authors.forename: I
authors.initials: I
authors.lastname: Nonaka
mesh_terms: D002675:Child, Preschool; D003574:Cytochrome c Group / Q000494:pharmacology; D004831:Epilepsies, Myoclonic / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology; D005073:Evoked Potentials, Somatosensory / Q000502:physiology*; D005260:Female; D005486:Flavin Mononucleotide / Q000494:pharmacology; D006801:Humans; D017243:MERRF Syndrome / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D010146:Pain / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D012008:Recurrence; D013788:Thalamus / Q000098:blood supply; D013831:Thiamine / Q000494:pharmacology; D015899:Tomography, Emission-Computed, Single-Photon
chemical_list: D003574:Cytochrome c Group; D005486:Flavin Mononucleotide; D013831:Thiamine