Fujimoto S, Mizuno K, Shibata H, Kanayama M, Kobayashi M, Sugiyama N, Ban K, Ishikawa T, Itoh T, Togari H, Wada Y
Department of Laboratory Medicine, Nagoya City University Medical School, Nagoya, Japan.
Pediatr Neurol. 1999 Jan;20(1):43-8. doi: 10.1016/s0887-8994(98)00088-5.
To clarify the electroencephalographic characteristics of mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS), the medical records and electroencephalograms of six patients with MELAS and two of their relatives with MELA (mitochondrial myopathy, encephalopathy, and lactic acidosis, without strokelike episodes) were retrospectively reviewed. All have a point mutation in the mitochondrial DNA at nucleotide position 3243. The electroencephalograms (n = 79) were divided into four groups according to the time relation to the strokelike episode: (1) before the first strokelike episode, (2) within 5 days after the strokelike episode (acute stage), (3) between 6 days and 1 month after the strokelike episode (subacute stage), and (4) more than 1 month after the strokelike episode (chronic stage). In the acute stage, 10 of the 11 electroencephalograms (9 strokelike episodes in four patients) revealed focal high-voltage delta waves with polyspikes (FHDPS), which were recognized as ictal electroencephalogram. Ictal events during FHDPS included focal clonic or myoclonic seizure and migrainous headache. In the subacute and chronic stages, focal spikes or sharp waves and 14- and 6-Hz positive bursts were frequently recorded. The authors' results suggest that FHDPSs present a reliable and accurate indicator of a strokelike episode in patients with MELAS.
为明确线粒体肌病、脑病、乳酸性酸中毒和卒中样发作（MELAS）的脑电图特征，我们回顾性分析了6例MELAS患者及其2例患有MELA（线粒体肌病、脑病和乳酸性酸中毒，无卒中样发作）亲属的病历和脑电图。所有患者线粒体DNA第3243位核苷酸均存在点突变。根据与卒中样发作的时间关系，将脑电图（n = 79）分为四组：（1）首次卒中样发作前；（2）卒中样发作后5天内（急性期）；（3）卒中样发作后6天至1个月（亚急性期）；（4）卒中样发作后1个月以上（慢性期）。在急性期，11份脑电图中的10份（4例患者出现9次卒中样发作）显示局灶性高电压δ波伴多棘波（FHDPS），被认为是发作期脑电图。FHDPS期间的发作事件包括局灶性阵挛或肌阵挛发作以及偏头痛性头痛。在亚急性期和慢性期，经常记录到局灶性棘波或尖波以及14和6赫兹正相暴发。作者的结果表明，FHDPS是MELAS患者卒中样发作的可靠且准确的指标。
