All four listed entities are pregnancy-associated and every one can produce this presentation, so no single option can be preferred. She is one week postpartum from a pre-eclamptic pregnancy, with intractable headache, a witnessed tonic-clonic seizure, a blood pressure of 240/100 and depressed arousal. Eclampsia is the label her history most directly supports, since a seizure superimposed on pre-eclampsia defines it. Posterior reversible encephalopathy syndrome is the usual imaging correlate of eclampsia and presents with exactly this triad. Reversible cerebral vasoconstriction syndrome, historically called postpartum angiopathy, shares the presentation, and cerebral venous sinus thrombosis peaks 7 to 14 days postpartum with the same features.
The overlap is biological, not merely a matter of naming. The peripartum state combines endothelial dysfunction driven by circulating antiangiogenic factors with a hypercoagulable state and abrupt swings in blood pressure. In eclampsia, failure of cerebral autoregulation at very high pressures together with endothelial injury allows plasma to leak into the interstitium, producing vasogenic edema that is parieto-occipital predominant because the posterior circulation has sparser sympathetic innervation and less autoregulatory reserve. In reversible cerebral vasoconstriction syndrome, segmental arterial narrowing produces thunderclap headache, convexity subarachnoid blood and infarction; in venous thrombosis, outflow obstruction causes often-hemorrhagic venous infarction. Roughly half of women with eclampsia show the imaging changes of posterior reversible encephalopathy syndrome.
The principle to carry away is that in the puerperium these diagnoses form a spectrum rather than mutually exclusive alternatives, so the imaging protocol should interrogate all of them: parenchymal MRI, MR venography and arterial imaging. Knowing this differential is a Core expectation for anyone taking neurology call, and because the item asks which diseases explain a clinical picture it sits on the Clinical aspects of neurologic disease axis.
Incorrect Answers
- A. Cerebral venous sinus thrombosis must be excluded, but selecting it alone discards the pre-eclamptic history and the extreme hypertension, and the lead-in asks which conditions may explain the presentation. Choose this if the headache had built over several days with papilledema and MR venography showed absent flow in a dural sinus.
- B. Eclampsia is the best single clinical label for this woman, which is exactly why it cannot be the best answer to a question asking which conditions are associated with pregnancy and may explain her presentation. Choose this if the lead-in had asked for the single most likely diagnosis in a woman with pre-eclampsia who has now seized.
- C. Posterior reversible encephalopathy syndrome is a clinical-radiological syndrome rather than an independent disease, and here it would be the manifestation of eclampsia. Choose this if MRI had shown symmetric parieto-occipital vasogenic edema with normal sinuses and arteries and you were asked to name the imaging syndrome.
- D. Reversible cerebral vasoconstriction syndrome is plausible in the first postpartum week, but no thunderclap onset is described and no vascular imaging is reported, so it cannot be singled out. Choose this if her headache had peaked within seconds and CTA had shown multifocal beaded narrowing that reversed at twelve weeks.
Testing Pearls
- Eclampsia, posterior reversible encephalopathy syndrome, reversible vasoconstriction and venous thrombosis all cause peripartum headache with seizures and can coexist.
- About half of women with eclampsia show the imaging changes of posterior reversible encephalopathy syndrome.
- Cerebral venous thrombosis peaks 7 to 14 days postpartum; postpartum arterial stroke clusters around the eighth day after delivery.
References
- Jankovic J, Mazziotta JC, Newman NJ, Pomeroy SL, editors. Bradley and Daroff's Neurology in Clinical Practice. 8th ed. Philadelphia, PA: Elsevier; 2022. p. 2072-2073.
- Feske SK, Singhal AB. Cerebrovascular disorders complicating pregnancy. Continuum (Minneap Minn). 2014;20(1 Neurology of Pregnancy):80-99.
- Fugate JE, Rabinstein AA. Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. Lancet Neurol. 2015;14(9):914-25.