Funakawa I, Mukai K, Terao A, Kawashima S, Mori T
Department of Internal Medicine, Kawasaki Medical School.
Rinsho Shinkeigaku. 1994 Oct;34(10):1052-4.
We report a case of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) associated with prosopagnosia, topographical disorientation, and periodic lateralized epileptiform discharge (PLED) on electroencephalography (EEG) in a 23-year-old right-handed man. The first MELAS attack occurred on March 1, 1991, while the patient was drinking. Magnetic resonance imaging (MRI) revealed a lesion of abnormal intensity in the left occipital lobe. The second attack occurred on October 1, 1991. This time, the major symptoms were visual loss of acute onset, nausea, and vomiting. EEG examination showed transient PLED. MRI revealed a new area of abnormal intensity in the right occipital lobe, lingual gyrus, fusiform gyrus and the posterior part of the parahippocampal gyrus. During the clinical course of the patient, prosopagnosia and topographical disorientation appeared. There have been few reports of MELAS associated with prosopagnosia, topographical disorientation, and PLED. However, MELAS attacks tend to occur in the cortex of the occipital lobe. We therefore believe that these neuropsychological symptoms and PLED are likely to be associated with MELAS.
我们报告了一例线粒体肌病、脑病、乳酸性酸中毒及卒中样发作（MELAS）病例，该病例发生在一名23岁右利手男性身上，伴有面孔失认症、地形定向障碍，且脑电图（EEG）显示有周期性一侧性癫痫样放电（PLED）。首次MELAS发作于1991年3月1日，当时患者正在饮酒。磁共振成像（MRI）显示左侧枕叶有异常信号病变。第二次发作于1991年10月1日。此次主要症状为急性发作的视力丧失、恶心和呕吐。EEG检查显示短暂性PLED。MRI显示右侧枕叶、舌回、梭状回及海马旁回后部有新的异常信号区域。在患者的临床病程中，出现了面孔失认症和地形定向障碍。很少有关于MELAS与面孔失认症、地形定向障碍及PLED相关的报道。然而，MELAS发作往往发生在枕叶皮质。因此，我们认为这些神经心理学症状和PLED可能与MELAS有关。
