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Show Cerebral Vasculopathies Masquerading as ;ia Michael W. Varner, MD See related case reports on pages 442 and 446. Dr. Varner is from the Department of Obstetrics and Gynecology at the University of Utah, Salt Lake City, Utah; e-mail: mvarner@hsc.utah.edu. © 2006 by The American College of Obstetricians and Gynecologists. Published by Lippincott Williams & Wilkins. ISSN: 0029-7844/06 A Ithough the randomized controlled trial remains the "gold standard" /Yfor clinical advancement of medical knowledge, important advances have historically been made by observant practitioners in the form of case reports and small studies. This issue of the Journal contains 2 informative case reports from groups in New York1 and Germany2 describing patients with postpartum cerebral vasculopathy presenting as delayed postpartum eclampsia. Obstetricians know that preeclampsia-eclampsia is a syndrome and is a final common pathway for multiple causes. It is also possible that other conditions, not always directly related to pregnancy, can mimic preeclampsia-eclampsia. One such entity is cerebral vasculopathy, an entity characterized by acute-onset headache, altered mental status, visual loss and seizures, and a diagnostic magnetic resonance imaging picture. This syndrome, like many other conditions, can masquerade as preeclampsia- eclampsia in pregnant and puerperal women but often has uncharacteristic or atypical features. Many individuals (this syndrome is not gender specific) have little or no proteinuria and they often have prominent central nervous system findings. Both of the patients described in the aforementioned reports had had no suggestion of preeclampsia- eclampsia during pregnancy or delivery and had ncw-onsct sudden severe headache and hypertension without proteinuria followed by seizures and persistent neurologic signs at 4 and 1 days postpartum, respectively. The cranial magnetic resonance imaging (MRI) findings in postpartum cerebral vasculopathy can vary and often result in this syndrome receiving different names. The posterior reversible encephalopathy syndrome typically has lesions predominantly in the posterior white matter, with some involvement of the overlying cortex. The lesions are hyperintense on Trwcightcd images and often seen on diffusion-weighted images, characteristic of vasogenic edema. Autopsy cases often demonstrate arteriolar fibrinoid necrosis with microinfarcts and no brain edema. However, a brain biopsy in a patient who survived this diagnosis revealed edematous white matter with no evidence of vessel wall damage or infarction, suggesting that the MRI images characteristically seen in this syndrome actually represent vasogenic edema and that autopsy changes may be an epiphenomenon/ In contrast, the MRI findings in Call-Fleming syndrome are primarily cortical in location. Individuals with Call-Fleming syndrome may have no obvious abnormalities on a standard MRI but will have characteristic angiopathic findings on magnetic resonance angiopathy. Indeed, a magnetic resonance angiopathy examination should be considered in such cases when the MRI is normal. Besides masquerading as preeclampsia-eclampsia, postpartum cere- VOL. 107, NO. 2, PART 2, FEBRUARY 2006 OBSTETRICS & GYNECOLOGY 437 Copyright© American College of Obstetricians and Gynecologists ( bral vasculopathy can be seen in a number of other conditions including hypercalcemia, uremia, and porphyria. It is also seen in the course of medication administration, particularly cytotoxic agents, immunosuppressants, intravenous immunoglobulin, vasoconstrictors, serotonergic drugs, and migraine medications. It is important to emphasize that the 2 entities described in these case reports-posterior reversible leukoencephalopathy syndrome and Call-Fleming syndrome-are difficult if not impossible to distinguish clinically and can both be associated with permanent neurologic deficits, or even death, if not diagnosed and treated promptly. It is possible that they are different manifestations of similar underlying pathophysiology. Prompt diagnosis and treatment with antihypertensives and anticonvulsants as well as treatment of any associated disorders and removal of any potential offending medications is essential because early treatment can prevent what might otherwise be progression to irreversible brain damage. Likewise, the findings are not always confined to the posterior regions of the brain, and they can affect both white and gray matter. In any case, it remains unknown why some individuals develop it and others don't. It is likely that the evolving and intertwined sciences of genomics, proteomics, and metabolomics will eventually provide major insights into the prediction of this syndrome: who will and who will not develop it, its likely severity, and its underlying mechanism(s). Until then, practitioners must remain vigilant and remember that women who develop this syndrome can have any number of underlying causes. Delayed postpartum preeclampsia has been recognized for many years. By definition it is atypical and thus warrants a careful search for alternative explanations. As obstetricians we are trained to consider alternatives to preeclampsia-eclampsia, such as dural sinus thrombosis, intracranial hemorrhage, and central nervous system infection or vasculitis. To this differential diagnosis we must now add postpartum cerebral vasculopathy. These 2 reports raise several important clinical points. First, the diagnosis of postpartum cerebral vasculopathy should be considered in any puerperal or pregnant woman with delayed postpartum or otherwise atypical preeclampsia-eclampsia. Central nervous system imaging should be considered promptly if the case seems atypical or if there are any localizing or persistent deficits. Second, treatment with mannitol and betamethasone should be initiated promptly to reduce intracranial pressure and with magnesium sulfate to prevent or treat convulsions. Failure to treat this syndrome promptly increases the risk of irreversible cerebral herniation. Third, as documented in the New York case,1 it is important to remember that there is nothing magic about discontinuing magnesium sulfate after a 24-hour interval. If a woman remains symptomatic after 24 hours of magnesium sulfate therapy, it should be continued until such time as she is improved. Likewise, appropriate longer-term anticonvulsant therapy may also be necessary. Finally, prompt control of blood pressure is mandatory in women with postpartum cerebral vasculopathy. Either /3-blockers or calcium channel blockers are appropriate antihypertensive agents. One of the great adventures of clinical medicine is that we're continually learning from our patients. The 2 women described in these case reports have taught us another valuable consideration in the complex syndrome we clinically describe as postpartum preeclampsia-eclampsia. I appreciate the efforts of the authors to bring this point to our attention and I'm glad that both women recovered completely. REFERENCES 1. Belogolovkin V, Levine SR, Fields MC, Stone JL. Postpartum eclampsia complicated by reversible cerebral herniation. Obstet Gynecol 2006;107:442-5. 2. Neudecker S, Stock K, Krasnianski M. Call-Fleming postpartum angiopathy in the puerperium: a reversible cerebral vasoconstriction syndrome. Obstet Gynecol 2006;107:446-9. 3. Schiff 1), Lopes MB. Neuropathological correlates of reversible posterior leukoencephalopathy. Neurocrit Care 2005;2:303-5. 438 Varner Cerebral Vasculopathies OBSTETRICS & GYNECOLOGY Copyright© American College of Obstetricians and Gynecologists (~^4 |
