Postpartum angiopathy with reversible posterior leukoencephalopathy.
BACKGROUND
Postpartum angiopathy (PPA) is a cerebral vasoconstriction syndrome of uncertain cause that affects large and medium-sized cerebral arteries. Postpartum angiopathy is frequently complicated by ischemic stroke. The reversible posterior leukoencephalopathy syndrome (RPLS) is a distinct clinical-radiological entity characterized by transient vasogenic edema on brain imaging. The pathophysiological features of RPLS are related to small-vessel dysfunction and breakdown of the blood-brain barrier.


OBJECTIVE
To report the coexistence of PPA and RPLS in 4 patients and to discuss possible interrelationships between these 2 entities.


METHODS
Four case reports and a review of the literature.


RESULTS
Four women developed a clinical-radiological syndrome overlapping PPA and eclampsia shortly after an uncomplicated pregnancy. All had acute severe ("thunderclap") headaches and hypertension. Three developed seizures. All patients had reversible angiographic narrowing of large and medium-sized cerebral arteries. Serial magnetic resonance imaging showed transient nonischemic brain lesions, resembling the lesions described in patients with RPLS. The results of extensive tests for cerebral vasculitis were negative.


CONCLUSIONS
These cases, and the literature, suggest an interrelationship between RPLS and cerebral vasoconstriction syndromes such as PPA.
Singhal
Aneesh B
AB
Stroke Service, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston, 02114, USA. asinghal@partners.org
D000328:Adult; D001921:Brain / Q000473:pathology; D001927:Brain Diseases / Q000150:complications* / Q000473:pathology; D001929:Brain Edema / Q000150:complications* / Q000473:pathology; D002533:Cerebral Angiography / Q000379:methods; D002536:Cerebral Arteries / Q000473:pathology; D015140:Dementia, Vascular / Q000150:complications* / Q000473:pathology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D049590:Postpartum Period; D012196:Review Literature as Topic; D013997:Time Factors; D014057:Tomography, X-Ray Computed / Q000379:methods; D014652:Vascular Diseases / Q000150:complications* / Q000473:pathology