Clinical evaluation of Reversible Posterior Leukoencephalopathy Syndrome in Korean Children: Multicenter Study / 대한소아신경학회지

ABSTRACT

PURPOSE: Reversible posterior leukoencephalopathy syndrome(RPLS) is a complex condition affecting gray/white matter of parieto-occipital lobes from multiple and different etiologies. This study was aimed to analyze clinical characteristics of this condition in Korean children. METHODS: A total of 36 patients from tertiary care medical centers were involved in the study. They were diagnosed as RPLS mainly by clinical and radiological manifestations. Their medical records and radiological features of brain MRI were retrospectively analyzed. RESULTS: Thirty six patients were involved in the study(21 males and 15 females, mean age 9.8+/-4.1 years of age). Main underlying causes included drug induced(47%), renal diseases(28%), neurogenic tumors(8%) and so on. Initial manifestations were seizures, headache, visual disturbance, mental change, and nausea/vomiting. 13 patients(36%) had two or more symptoms. The brain MRI showed typical pattern of fairly symmetric, high T2 signal intensity in both parieto-occipital regions in 23 patients(64%) and various, atypical pattern in 13 patients(36%). 26 patients had the lesions affecting predominantly white matter, but 10 patients had the lesions affecting predominantly gray matter. 34 patients(94 %) had bilateral lesions, but 2 patients(6%) had unilateral lesion. This complex syndrome was associated with acute rise of blood pressure from a variety of conditions. A patient ended up a visual deficit despite the good prognosis. CONCLUSION: RPLS in Korean children seems to be complex, but a better understanding of this complex syndrome will lead to better clinical outcome by avoiding unnecessary investigations and appropriate management.