Received 19 May 2010, accepted 15 June 2010 | Dimitri Renard, Pierre Labauge, Rik Vandenberghe
Reversible posterior leukoencephalopathy syndrome (RPLS) is a condition characterized by subacute onset of headache, decreased alertness, vomiting, seizures, and visuoperceptual disturbances, with bilateral white matter lesions in the posterior brain regions on MRI. It is commonly associated with arterial hypertension and immunosuppressive or cytotoxic treatments. MRI findings, such as hyperintensities in parietooccipital white matter on T2, FLAIR, and ADC sequences, help differentiate RPLS from ischemic events. The pathophysiology is likely vasogenic edema, with factors like impaired cerebral autoregulation, endothelial dysfunction, and vasospasm contributing. Treatment involves discontinuing the causative drug, managing hypertension, and antiepileptic therapy. Early treatment leads to clinical and radiological recovery, but delayed treatment can result in irreversible brain damage. RPLS can occur in children, especially those with leukemia, and is the most common cause of seizures in such cases. Risk factors include drugs like cyclosporine, hypertension, and certain infections. Differential diagnosis includes conditions like ischemic stroke, CNS vasculitis, and PML. MRI is crucial for diagnosis, with T2, FLAIR, and ADC sequences showing characteristic findings. Prognosis is generally good with early treatment, but poor outcomes are associated with extensive lesions, ischemia, or hemorrhage. Future research aims to better understand the pathophysiology and risk factors for RPLS, particularly in the context of increasing use of cytotoxic and immunosuppressive drugs.Reversible posterior leukoencephalopathy syndrome (RPLS) is a condition characterized by subacute onset of headache, decreased alertness, vomiting, seizures, and visuoperceptual disturbances, with bilateral white matter lesions in the posterior brain regions on MRI. It is commonly associated with arterial hypertension and immunosuppressive or cytotoxic treatments. MRI findings, such as hyperintensities in parietooccipital white matter on T2, FLAIR, and ADC sequences, help differentiate RPLS from ischemic events. The pathophysiology is likely vasogenic edema, with factors like impaired cerebral autoregulation, endothelial dysfunction, and vasospasm contributing. Treatment involves discontinuing the causative drug, managing hypertension, and antiepileptic therapy. Early treatment leads to clinical and radiological recovery, but delayed treatment can result in irreversible brain damage. RPLS can occur in children, especially those with leukemia, and is the most common cause of seizures in such cases. Risk factors include drugs like cyclosporine, hypertension, and certain infections. Differential diagnosis includes conditions like ischemic stroke, CNS vasculitis, and PML. MRI is crucial for diagnosis, with T2, FLAIR, and ADC sequences showing characteristic findings. Prognosis is generally good with early treatment, but poor outcomes are associated with extensive lesions, ischemia, or hemorrhage. Future research aims to better understand the pathophysiology and risk factors for RPLS, particularly in the context of increasing use of cytotoxic and immunosuppressive drugs.