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

doi:10.1016/S0387-7604(96)00558-X

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

Pediatrics

Research output: Contribution to journal › Article › peer-review

12 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 language	English
Pages (from-to)	205-208
Number of pages	4
Journal	Brain and Development
Volume	19
Issue number	3
DOIs	https://doi.org/10.1016/S0387-7604(96)00558-X
Publication status	Published - 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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10.1016/S0387-7604(96)00558-X

Cite this

Tanaka, S., Osari, S. I., Ozawa, M., Yamanouchi, H., Goto, Y. I., Matsuda, H., & Nonaka, I. (1997). 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. Brain and Development, 19(3), 205-208. https://doi.org/10.1016/S0387-7604(96)00558-X

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. / Tanaka, Soichiro; Osari, Shin Ichi; Ozawa, Matsuko et al.

In: Brain and Development, Vol. 19, No. 3, 04.1997, p. 205-208.

Research output: Contribution to journal › Article › peer-review

Tanaka, S, Osari, SI, Ozawa, M, Yamanouchi, H, Goto, YI, Matsuda, H & Nonaka, I 1997, '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', Brain and Development, vol. 19, no. 3, pp. 205-208. https://doi.org/10.1016/S0387-7604(96)00558-X

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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.",

keywords = "Central pain, Cytochrome c, MERRF, SPECT, Thalamic hypoperfusion",

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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

Keywords

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