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postpartum cerebral angiopathy ( pca ) is a cerebral vasoconstriction syndrome developing shortly after delivery , without signs of preceding eclampsia .
, we report on a closely monitored , uneventful pregnancy of a woman with a previous severe episode of pca .
a 32-year - old female patient was first treated at our university hospital when she developed a severe episode of pca after delivery of her second child .
1 ) , becoming symptomatic , with an acute onset of very severe headache 4 days after delivery , left - sided hemianopia , and generalized tonic - clonic seizures .
follow - up mra showed a clear resolution of vasoconstriction 7 and 15 days after diagnosis .
the patient was discharged without deficits , but was advised to have her status re - evaluated before a next pregnancy .
eight months after this first episode , the patient presented to our outpatient clinic being 6 weeks pregnant .
over the course of her pregnancy , no signs of vasoconstriction or epileptic activity were identified on repeated neurological examinations , eegs , or mris and transcranial doppler ultrasounds ( tcd ) .
after 40 weeks of pregnancy , our patient had an uncomplicated vaginal delivery of a healthy boy .
as during the second pregnancy , she had not received any medications having pca as a possible side effect .
daily tcd from 3 days before to 10 days after delivery did not reveal signs of vasoconstriction .
2 ) or signs of vasoconstriction were seen on mri . at follow - up 6 months later , the patient was well without complaints .
pca is considered a rare , possibly underdiagnosed , but serious neurological complication in delivering women [ 3 , 4 ] .
it is one of several reversible cerebral vasoconstriction syndromes ( rcvs ) , characterized by reversible vasoconstriction of the cerebral arteries .
rcvs are usually associated with an acute onset of thunderclap headache with or without neurologic deficits .
the pathophysiology of rcvs is thought to involve an acute change in cerebrovascular tone , which may be spontaneous or evoked by various exogenous or endogenous factors , with an uncertain pathophysiological mechanism [ 5 , 6 , 7 ] .
it is not clear whether vasospasm , vasculitis or another mechanism or factor is the underlying reason for pca [ 6 , 8 ] , but the pathophysiology may be similar to the disease mechanisms in eclampsia . since it is known that the risk of a repeated pre - eclampsia is more than twice as high as the risk in the general population , it is justified to consider that there is also an increased risk for repeated pca in patients with pca in a previous pregnancy . for eclampsia
, the rate of recurrence may be as high as 50% , but for rcvs in general , and pca in particular , these data are not available , especially since patients are typically advised to avoid the triggering factors . in a literature review
, we only found one report on the recurrence of pca where a patient had severe intracranial hemorrhages after two consecutive pregnancies . in comparison ,
our patient 's course of her third pregnancy is encouraging , as it demonstrates no recurrence of pca even after a previous severe episode of pca .
although conventional angiography is , by definition , the gold standard , non - invasive investigations such as mri and ct of the brain and cerebral vessels have been largely used to support the diagnosis and follow - up .
the results of the brain mri are frequently normal in rcvs but can reveal evidence of infarction as well as parenchymal hemorrhages and small non - aneurysmal subarachnoid hemorrhages on the cortical surface . furthermore , with the development of the mri technique , the quality of the mra rapidly becomes better .
therefore , patients with pregnancies following an episode of pca should be monitored closely . due to the very limited number of cases and the lack of studies ,
it is unclear what surveillance of the patients would make sense . of course , the basis for close monitoring are neurologic examinations on the days before and after delivery .
besides clinical monitoring , tcd seems an ideal tool to monitor patients for the development of cerebral vasoconstriction .
tcd is non - invasive , has no side effects , is easy to perform , and is cheap .
we chose to monitor our patient closely by tcd , checking for an increase of cerebral blood flow velocities monthly during the last 4 months of pregnancy and daily from 3 days before to 10 days after delivery .
furthermore , we recommend an mra and mri whenever clinical signs consistent with pca develop ( e.g. headaches , seizures , focal neurologic deficits ) .
since our patient had suffered from an epileptic seizure during pca , we also performed an eeg the week before and after delivery to rule out epileptiform discharges .
for the treatment of severe episodes of pca , calcium channel blockers are used , but only with little substantiating evidence [ 1 , 13 ] .
occurrence of subclinical signs of vasoconstriction on mra or tcd may warrant consideration of oral nimodipine even before the onset of clinical signs .
due to the lack of evidence , counseling patients with pca with respect to following pregnancies is difficult compared to other gestational diseases , but our report is encouraging compared to a previous report on recurrence of pca with most severe complications .
however , further studies are certainly needed to adequately understand the risk of a new pregnancy and to understand factors contributing to pca . until more comprehensive data on new pregnancies is available , close monitoring is recommended .
postpartum cerebral angiopathy ( pca ) is a cerebral vasoconstriction syndrome developing shortly after delivery , without signs of preceding eclampsia . 
 the risk for recurrence of pca is unknown . here 
 , we report on a closely monitored , uneventful pregnancy of a woman with a previous severe episode of pca . in summary 
 , this case report demonstrates that pca does not necessarily recur in following pregnancies , even after previous severe episodes .