Posterior Leukoencephalopathy Syndrome in Children and Adolescents

Abstract

Posterior leukoencephalopathy syndrome is a recently identified clinical and radiologic entity. The characteristic radiologic findings are bilateral gray and white matter edema in the posterior regions of the cerebral hemispheres. This article reports clinical and radiologic findings in 10 consecutive episodes of posterior leukoencephalopathy syndrome that were diagnosed in 9 children and adolescents. The causes were immunosuppressive therapy in 7 patients and a combination of renal failure and hypertension in 3. The most common presenting symptoms were seizure and altered consciousness; others included headache, sixth nerve palsy, and cortical blindness. Imaging demonstrated abnormalities in the parietal and occipital lobes in all 10 episodes. The signs and symptoms resolved after immunosuppressive agents were reduced or discontinued, or after uremia and hypertension were corrected. Four patients underwent follow-up cranial imaging, and the images showed nearly complete or complete resolution. The syndrome was clinically reversible in all patients.

Evaluation of nine children with reversible posterior encephalopathy syndrome

“…Changes in the gray matter, cerebellum, and brainstem have also been described. [1,8,11] MRI shows hypotense lesions within the posterior white-matter regions on T1, and hyperintense signal on T2-weighted and FLAIR images. DWI reveals increased diffusion in affected regions, suggesting vasogenic oedema in these areas.…”

Posterior reversible leukoencephalopathy syndrome in children with hematologic disorders

“…The most common factors are immunosuppressive drugs (cyclosporine, anti-thymocyte globulin, tacrolimus, rituximab, interferon), and chemotherapeutic agents (methotrexate, L-asparaginase, adriamycin, cyclophosphamide, cytosine arabinoside, vincristine) [2,4,7,[8][9][10][11]. Sickle cell disease, hypertension, acute blood pressure changes, renal failure, TLS, infection, sepsis, shock, and organ transplantation are some factors that can also cause PRES [3,5,[12][13][14][15]. The exact etiopathogenesis of posterior leukoencephalopathy syndrome is still unknown.…”

A case of reversible posterior leukoencephalopathy syndrome in a patient on peritoneal dialysis

“…The visual disorders are frequently associated with neuroradiological findings, predominantly white matter abnormalities of the parieto-occipital lobes. [1][2][3] Reported comorbid conditions include hypertension (53%), renal disease (45%), dialysis dependency (21%), malignancy (32%), and transplantation (24%).…”

“…8 Since the term 'RPLS' characterizes this disorder, RPLS is usually reversible within 2 weeks by immediate control of BP and discontinuation of immunosuppressive agents. 3 When the diagnosis and treatment of RPLS are delayed, cerebral hemorrhage or cerebral infarction may occur, and the symptoms become irreversible. 9 In this case, the patient was discharged with no neurological deficit because of early diagnosis and timely, appropriate therapy.…”

“…An abrupt BP increase in patients with renal disease has been reported to be a particularly significant cause of RPLS. [1][2][3] Although the pathogenesis of RPLS remains unclear, it appears to be related to disordered cerebral autoregulation and endothelial dysfunction. 4 Because of the heterogeneous nature of this syndrome, different mechanisms may be etiologically important in different clinical situations.…”