Tanaka S, Osari S, Ozawa M, Yamanouchi H, Goto Y, Matsuda H, Nonaka I
Department of Child Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders, Tokyo, Japan.
Brain Dev. 1997 Apr;19(3):205-8. doi: 10.1016/s0387-7604(96)00558-x.
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.
我们报告了一名患有肌阵挛性癫痫伴破碎红纤维（MERRF）的3岁女孩，她反复出现身体右侧疼痛发作和感觉异常。尽管磁共振成像显示丘脑信号强度正常，但单光子发射计算机断层扫描（SPECT）显示丘脑灌注不足。体感诱发电位显示早期皮层反应延迟，尤其是右侧正中神经刺激时。静脉注射细胞色素c、黄素单核苷酸和硫胺二磷酸可消除难以忍受的疼痛。SPECT结果和神经生理学发现客观地支持了这种临床改善。这些观察结果表明，丘脑功能障碍是她疼痛的原因，细胞色素c治疗对这种症状有益。
