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Vivien H. Lee, MD; Eelco F. M. Wijdicks, MD; Edward M. Manno, MD; Alejandro A. Rabinstein, MD

Arch Neurol. 2008;65(2):205-210.

Background  Reversible posterior leukoencephalopathy syndrome (RPLS) is characterized by neuroimaging findings of reversible vasogenic subcortical edema without infarction. The clinical syndrome of RPLS typically involves headache, encephalopathy, visual symptoms, and seizures.

Objective  To retrospectively identify patients with RPLS with a characteristic clinical presentation and neuroimaging abnormalities and documented improvement on repeated neuroimaging.

Design  Retrospective.

Setting  Mayo Clinic.

Patients  Thirty-six patients with RPLS.

Main Outcome Measures  Associated comorbid medical conditions, presenting clinical symptoms, duration of clinical symptoms, diagnostic test results (magnetic resonance imaging, electroencephalography, and lumbar puncture), and time to clinical and neuroimaging recovery.

Results  We identified 38 episodes of RPLS in 36 patients (20 females and 16 males) with a mean age of 44.7 years. Comorbid conditions included hypertension (53%), renal disease (45%), dialysis dependency (21%), malignancy (32%), and transplantation (24%). Presenting symptoms included clinical seizures (87%), encephalopathy (92%), visual symptoms (39%), and headache (53%). Mean peak systolic blood pressure at presentation was 187 mm Hg. Clinical symptoms resolved after a mean of 5.3 days. Atypical neuroimaging features included significant frontal involvement in 22 episodes (58%), gray matter lesions in 16 (42%), unilateral lesions in 2 (5%), hemorrhage in 2 (5%), recurrent RPLS in 2 (5%), confluent lesions in 2 (5%), and foci of permanent injury in 10 (26%). Twenty-two episodes (58%) had brainstem/cerebellar involvement on neuroimaging.

Conclusions  This is the largest clinical series to date of RPLS with confirmed neuroimaging improvement. Clinical recovery occurred in most patients within days. The condition was rarely isolated to the parieto-occipital white matter, and atypical neuroimaging features were frequent.

Author Affiliations: Section of Cerebrovascular Disease and Neurological Critical Care, Department of Neurological Sciences, Rush University Medical Center, Chicago, Illinois (Dr Lee); and Division of Critical Care Neurology, Department of Neurology, Mayo Clinic College of Medicine, Rochester, Minnesota (Drs Wijdicks, Manno, and Rabinstein).

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