Spectrum and potential pathogenesis of reversible posterior leukoencephalopathy syndrome

J Stroke Cerebrovasc Dis. 2012 Nov;21(8):873-82. doi: 10.1016/j.jstrokecerebrovasdis.2011.05.010. Epub 2011 Jun 23.

Abstract

Background: Controversy still exists over the etiology and pathophysiology of reversible posterior leukoencephalopathy syndrome (RPLS). This large single-center case series aims to describe the clinical and imaging features of RPLS in an attempt to deduce the etiology of the disorder and the mechanisms of brain injury.

Methods: A retrospective chart and imaging review was conducted on 59 cases of RPLS in 55 patients.

Results: Five RPLS imaging patterns were observed: posterior predominant (n = 40), anterior predominant (n = 7), diffuse lesion (n = 7), basal ganglia predominant (n = 3), and brainstem/cerebellum predominant patterns (n = 2). RPLS resulted in permanent neurologic deficits in 14 patients and death in 4 patients. Hypertension was seen in 57 (97%) cases, and mean arterial blood pressure exceeded 140 mm Hg in 30 (51%) cases. Follow-up magnetic resonance imaging scans revealed a significant worsening of vasogenic edema in 2 cases, both with persistent hypertension. Magnetic resonance imaging scans revealed areas of ischemia in 14 cases, all within or at areas closely adjacent to vasogenic edema. Diffuse vasculopathy was seen in 8 cases. There was a lack of correlation between the presence of vasculopathy and the degree of vasogenic edema (P = .62), but a correlation was suggested between ischemia and vasculopathy (P = .02).

Conclusions: This study strongly suggests that hypertension-induced vasodilation rather than vasoconstriction-mediated hypoxia is likely the major mechanism responsible for the development of vasogenic edema, and that vasoconstriction may contribute to the development of ischemia in RPLS.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Antineoplastic Agents / adverse effects
  • Arterial Pressure
  • Brain / pathology
  • Brain Edema / classification
  • Brain Edema / diagnosis*
  • Brain Edema / etiology
  • Brain Edema / pathology
  • Brain Edema / physiopathology
  • Brain Ischemia / diagnosis
  • Brain Ischemia / etiology
  • Cerebral Angiography / methods
  • Diffusion Magnetic Resonance Imaging
  • Female
  • Humans
  • Hypertension / complications
  • Hypertension / diagnosis
  • Hypertension / physiopathology
  • Immunosuppressive Agents / adverse effects
  • Magnetic Resonance Angiography
  • Male
  • Middle Aged
  • Pennsylvania
  • Posterior Leukoencephalopathy Syndrome / classification
  • Posterior Leukoencephalopathy Syndrome / diagnosis*
  • Posterior Leukoencephalopathy Syndrome / etiology
  • Posterior Leukoencephalopathy Syndrome / pathology
  • Posterior Leukoencephalopathy Syndrome / physiopathology
  • Predictive Value of Tests
  • Prognosis
  • Retrospective Studies
  • Risk Factors
  • Time Factors
  • Tomography, X-Ray Computed
  • Ultrasonography, Doppler, Transcranial
  • Vasoconstriction
  • Vasodilation
  • Young Adult

Substances

  • Antineoplastic Agents
  • Immunosuppressive Agents