Reversible Cerebral Vasoconstriction Syndrome (RCVS) is a rare condition characterized by thunderclap headache and reversible vasoconstriction of the cerebral arteries. It is a cerebrovascular disorder caused by a transient disregulation of cerebral vascular tone, leading to multifocal arterial constriction and dilation. Most cases mainly occur during postpartum period or after exposure to vasoactive substances. The primary clinical manifestation is recurrent sudden-onset and severe headache over 1–3 weeks, often accompanied by nausea, vomiting, photophobia, confusion and blurred vision. The major complications are subarachnoid hemorrhage and stroke.1,2 Incidence is unknown but it does not appear rare on the basis of prospective and retrospective studies.3
Posterior Reversible Encephalopathy Syndrome (PRES) is a syndrome characterized by reversible posterior brain vasogenic edema, most commonly in the parieto-occipital regions. It's caused by a rapid increase of arterial blood pressure that can lead to cerebral hyperperfusion with consequent vascular leakage and vasogenic edema. It seems to be triggered by sympathetic hyperactivity and endothelial disfunction caused by circulating endogenous or exogenous substances that might be responsible of endothelial activation resulting in release of immunogenic and vasoactive substances. Neurological symptoms, including headache, visual deficits, disorders of consciousness, confusion, seizures and focal neurological signs are appreciated in this condition as well. The incidence of PRES in pregnancy or puerperium is not known, eventhough this syndrome is increasingly diagnosed. 4,5
These two syndromes share endothelial dysfunction as a pathophysiological feature.6, 7
Scientific literature about coexistence of PRES-RCVS-post dural puncture headache (PDPH) is poor and limited to case reports.8–12 This reports describe cases of women who had a history of migraine, preeclampsia or in good clinical conditions that underwent Cesarean delivery (C-delivery) with spinal or epidural anesthesia with inadvertent dural puncture. They presented a severe headache between day 2 and 7 after Cesarean section (C-section), and alteration of the consciousness status between day 3 and 7. The diagnosis was reached only after imaging13–15. Soon afther that, medication that comprehend antiepileptic drugs (Levetiracetam, Phenytoin, MgSO4) and Nimodipin for the vasospasm, was started. All this papers try to find a correlation between dural puncture and RCVS-PRES, and they all agree in suggesting that dural puncture could be a possible trigger in a substrate of disorders of the cerebral vascular function.
We present a case of RCVS-PRES in a postpartum woman with inadvertent dural puncture during epidural analgesia catether positioning. We point the attention on clinical characteristics and timing of the headache as fundamental data to have a prompt diagnosis and treatment. Then we consider the possibility for dural puncture to be a trigger for RCVS-PRES.