A fatal case of postpartum cerebral angiopathy with literature review
- First Online:
Postpartum cerebral angiopathy (PCA) is a rare and pathophysiologically ill-characterized cerebral vasoconstriction syndrome, occurring within 30 days of a usually uncomplicated pregnancy and delivery. Its onset has been associated with the use of vasoactive medications, particularly ergot alkaloids. Other cases have occurred in the absence of these medications, prompting conjecture into possible overlap between PCA and other conditions known to cause cerebral vasoconstriction, including primary angiitis of the central nervous system and postpartum eclampsia. The vast majority of cases follow a relatively benign course; however, a fatal case has been reported. Histopathologic findings in PCA, so far limited to the fatal case and two more recent biopsies, have been nonspecific.
Here we present a second fatal case of PCA, including pre- and post-mortem histopathologic analysis. We also include a review of all PCA cases reported in the English literature.
Criteria for the clinical diagnosis of PCA are proposed and used to select case reports from the medical literature. Data pertaining to patient characteristics, clinical symptomatology, cerebral imaging findings, and clinical outcomes are compared between cases associated with the postpartum use of vasoactive medications and spontaneous cases.
We conclude that histopathologic findings in PCA are nonspecific and secondary to ischemic brain injury. Functional vasoconstriction is the most likely primary pathophysiologic process in PCA. The etiology in cases associated with medications may be due to idiosyncratic reactions to these agents. Significant overlap in symtomatology and clinical features exists between spontaneous cases and late postpartum eclampsia.
- Calabrese LH, Mallek JA (1988) Primary angiitis of the central nervous system. Report of 8 new cases, review of the literature, and proposal for diagnostic criteria. Medicine (Baltimore) 67:20–39Google Scholar
- Ellison C, Martens R, Belkin R, Bourdette D (1988) Postpartum cerebral angiopathy: A benign variant of isolated CNS vasculitis? Neurolo 38:110–110Google Scholar
- Gary Cunningham, Kenneth J. Leveno, Steven L. Bloom, John C. Hauth, Larry C. Gilstrap, Katharine D. Wenstrom. 2005. Williams Obstetrics. 1600Google Scholar
- Iffy L, Lindenthal J, McArdle JJ, Ganesh V (1996a) Severe cerebral accidents postpartum in patients taking bromocriptine for milk suppression. Isr J Med Sci 32:309–312Google Scholar
- Iffy L, McArdle JJ, Ganesh V (1996b) Intracerebral hemorrhage in normotensive mothers using bromocriptine postpartum. ZentralblGynakol 118:392–395Google Scholar
- Leitch CR, Cameron AD, Walker JJ (1997b) The changing pattern of eclampsia over a 60-year period. Br J Obstet Gynaecol 104:917–922Google Scholar
- Newbould S (2002) Postpartum eclampsia. Am Fam Physician 66:1Google Scholar
- No authors listed (1984) Postpartum hypertension, seizures, strokes reported with bromocriptine. FDA Drug Bul 14:3–4Google Scholar
- Ringer AJ, Qureshi AI, Kim SH, Fessler RD, Guterman LR, Hopkins LN (2001) Angioplasty for cerebral vasospasm from eclampsia. Surg Neurol 56:, 73–8; discussion 378–379Google Scholar
- Singhal AB (2004a) Cerebral vasoconstriction syndromes. Top Stroke Rehabil 11:1–6Google Scholar
- Song JK, Fisher S, Seifert TD, Cacayorin ED, Alexandrov AV, Malkoff MD, Grotta JC, Campbell MS.(2004) Postpartum cerebral angiopathy: atypical features and treatment with intracranial balloon angioplasty. NeuroradiologyGoogle Scholar
- Ursell MR, Marras CL, Farb R, Rowed DW, Black SE, Perry JR (1995) Recurrent intracranial hemorrhage due to postpartum cerebral angiopathy: implications for management. Stroke 29:1995–1998Google Scholar