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.
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.
Tanaka
S
S
Department of Child Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders, Tokyo, Japan.
Osari
S
S
Ozawa
M
M
Yamanouchi
H
H
Goto
Y
Y
Matsuda
H
H
Nonaka
I
I
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
D003574:Cytochrome c Group; D005486:Flavin Mononucleotide; D013831:Thiamine