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

Soichiro Tanaka, Shin Ichi Osari, Matsuko Ozawa, Hideo Yamanouchi, Yu Ichi Goto, Hiroshi Matsuda, Ikuya Nonaka

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11 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)205-208
Number of pages4
JournalBrain and Development
Volume19
Issue number3
DOIs
Publication statusPublished - 1997 Apr

Keywords

  • Central pain
  • Cytochrome c
  • MERRF
  • SPECT
  • Thalamic hypoperfusion

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Developmental Neuroscience
  • Clinical Neurology

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