We reported the clinical and neuroradiological findings of 8 patients (4 males; 4 females; age range; 3 - 14 years) with posterior leukoencephalopathy syndrome (PLES). Previous case reports suggested that one of the major factors leading to PLES was severe hypertension. We divided the patients into two groups with or without severe hypertension, and each group was divided furthermore into two corresponding to the reversibility of brain lesions. The four cases of PLES with severe hypertension were all due to renal hypertension, and half of them resulted in irreversible outcomes, which were considered to be associated with inappropriate treatment. On the other hand, the four cases of PLES without severe hypertension showed reversible change, suggesting intravascular endothelial dysfuncton in respect of their causing factors. We consider that PLES could be caused without sever hypertension, particularly in children. Those with severe hypertension must be treated immediately to prevent irreversible brain damage.

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