From the Guidelines
Reversible Posterior Leukoencephalopathy Syndrome (RPLS) is a rare neurological disorder characterized by acute neurological deficits, including altered consciousness, visual disturbances, blindness, headaches, and seizures 1.
Key Characteristics
- The condition is often associated with hypertensive encephalopathy, which can lead to cerebral edema, microscopic hemorrhages, and infarctions 1.
- Magnetic resonance imaging (MRI) with FLAIR imaging can confirm the diagnosis by showing white matter lesions in the posterior regions of the brain 1.
Management
- Treatment is primarily symptomatic and involves discontinuation of offending medications, such as ifosfamide 1, and control of blood pressure.
- In some cases, anticonvulsants or antihypertensives may be necessary to manage symptoms, and hospitalization may be required for severe cases.
- The goal of treatment is to achieve spontaneous full remission without sequelae, which is possible in almost all cases with prompt and appropriate management 1.
From the Research
Definition and Characteristics of Reversible Posterior Leukoencephalopathy Syndrome (RPLS)
- RPLS is a rare neurological syndrome of the brain, causing symptoms such as headaches, seizures, altered mental status, and visual disturbances 2, 3, 4, 5, 6.
- The condition is predominantly associated with hypertension, eclampsia, renal impairment, cytotoxic drugs, immunosuppressive agents, and molecular targeted agents 2, 3, 4, 5, 6.
- RPLS is characterized by neuroimaging findings of reversible vasogenic subcortical edema without infarction 3.
- The clinical syndrome of RPLS typically involves headache, encephalopathy, visual symptoms, and seizures 3.
Clinical Presentation and Diagnosis
- Presenting symptoms of RPLS include clinical seizures, encephalopathy, visual symptoms, and headache 3.
- Comorbid conditions associated with RPLS include hypertension, renal disease, dialysis dependency, malignancy, and transplantation 3.
- Diagnostic tests used to diagnose RPLS include magnetic resonance imaging (MRI), electroencephalography, and lumbar puncture 2, 3, 5.
- Neuroimaging findings of RPLS may include attenuation abnormalities in the bilateral parietal region and the left occipital lobe, with suspected metastasis 2.
- Cranial T2-weighted MRI may indicate bilateral regions of increased signal intensity in the occipital, temporal, and periventricular white matter 2.
Treatment and Outcomes
- Treatment of RPLS typically involves anticonvulsants, steroids, and antihypertensive drugs 2, 5.
- Clinical recovery from RPLS can occur within days to weeks, with radiographic resolution occurring in the majority of patients 3, 6.
- However, some patients may suffer residual neurologic deficits or death 6.
- Early recognition and treatment of RPLS triggers are crucial to prevent permanent sequelae 6.