Oppenheim C, Galanaud D, Samson Y, Sahel M, Dormont D, Wechsler B, Marsault C
Department of Neuroradiology, Groupe Hospitalier Pitié-Salpêtrière, Paris VI University, 47 Boulevard de l'Hôpital, 75651 Paris, Cedex 13, France.
J Neurol Neurosurg Psychiatry. 2000 Aug;69(2):248-50. doi: 10.1136/jnnp.69.2.248.
The precise mechanism of neurological symptoms in patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) is still controversial. The diffusion weighted MR findings at the acute phase of a neurological event in MELAS are described and the pathophysiology of stroke-like lesion in the light of diffusion changes is discussed. Brain MRI was performed 2 days after the sudden onset of cortical blindness in a 25 year old patient with MELAS. Fluid attenuated inversion recovery (FLAIR) images showed multifocal cortical and subcortical hyperintensities located bilaterally in the frontobasal and the temporo-occipital lobes. Diffusion weighted images showed normal to increased apparent diffusion coefficient values in the acute left temporooccipital lesion and increased values in the older stroke-like lesions. These diffusion weighted findings support the metabolic rather than the ischaemic pathophysiological hypothesis for stroke-like episodes occurring in MELAS. Normal or increased apparent diffusion coefficient values within 48 hours of a neurological deficit of abrupt onset should raise the possibility of MELAS, especially if conventional MR images show infarct-like lesions.
线粒体肌病、脑病、乳酸酸中毒和卒中样发作(MELAS)患者神经症状的确切机制仍存在争议。本文描述了MELAS患者神经事件急性期的扩散加权磁共振成像(MR)表现,并根据扩散变化探讨了卒中样病变的病理生理学。对一名突发皮质盲2天后的25岁MELAS患者进行了脑部MRI检查。液体衰减反转恢复(FLAIR)图像显示双侧额底部和颞枕叶存在多灶性皮质和皮质下高信号。扩散加权图像显示急性左侧颞枕叶病变的表观扩散系数值正常或增加,而在较陈旧的卒中样病变中该值增加。这些扩散加权结果支持MELAS中卒中样发作的代谢而非缺血性病理生理假说。突发神经功能缺损48小时内表观扩散系数值正常或增加应提高MELAS的可能性,尤其是当传统MR图像显示梗死样病变时。