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

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 S; Osari S; Ozawa M; Yamanouchi H; Goto Y; Matsuda H; Nonaka I

Brain & Development

1997

Child; Female; Humans; Magnetic Resonance Imaging; Preschool; Q3 Literature Search; Emission-Computed; Tomography; Evoked Potentials; Epilepsies; Recurrence; Pain/diagnosis/drug therapy/etiology; Single-Photon; Somatosensory/physiology; Cytochrome c Group/pharmacology; Flavin Mononucleotide/pharmacology; MERRF Syndrome/complications/diagnosis/physiopathology; Myoclonic/complications/physiopathology/radionuclide imaging; Thalamus/blood supply; Thiamine/pharmacology

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.

1997

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