Background: Headache is one of the most common symptoms that are encountered in the postpartum period. We experienced a rare case of multiple phenomena of postpartum stroke occurred at the same time.
Case: A 38-year-old woman experienced sudden onset of thunderclap headache after delivery. We diagnosed cervicocephalic arterial dissection (CAD), and posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS) with cortical subarachnoid hemorrhage on the basis of clinical course and neuroradiological findings.
Conclusion: Our case was rare in that multiple phenomena of postpartum stroke occurred at the same time. What is remarkable about this case was the confirmation of the reversibility of these pathological conditions through multifaceted evaluations, including the MRI motion-sensitized driven equilibrium 3D turbo spin echo (MSDE) sequencing method.
Headache is one of the most common symptoms that are encountered in the postpartum period.1 It may be attributable to dehydration, sleep deprivation, preeclampsia, among other etiologies. In contrast, more serious and life-threatening conditions such as RCVS, SAH and CAD have been reported as causes of postpartum headache.2, 3, 4 We experienced a rare case of multiple phenomena of postpartum stroke occurred at the same time and so we present a case report and a review of the literature.
A 38-year-old woman had a normal pregnancy and gave birth to her second baby by vaginal delivery without preeclampsia or other complications. She had no significant medical history, and there was no family history of aneurysmal SAH or migraine. She had no history of taking medicine. She initially had severe stiff shoulder and then begun to experience sudden thunderclap headaches with epigastric pain on the 18th day after delivery. At the time of visiting our emergency department, her consciousness was clear and had no neurological deficits. The thunderclap headache and stiff shoulder improved. Laboratory investigations were all within normal limits, including blood cell count, liver enzyme levels, renal function and electrolytes and no coagulation abnormality. Urinalysis revealed no proteinuria.
The neuroradiological findings at the onset were shown in Figure 1. Computed tomography (CT, Aquilion PRIME, Canon Medical Systems Corporation) showed interhemispheric subarachnoid hemorrhage covering the cortical surface of the frontoparietal lobe (Fig. 1A). Magnetic resonance image (MRI, Achieva 3.0T TX Quasar, Philips) fluid-attenuated inversion recovery (FLAIR; repetition time (TR)=13000 ms, echo time (TE)= 140 ms, field of view (FOV)=210 mm, matrix size=512*512, slice thickness=6 mm, slice gap=0.6mm, NSA=1) revealed high-intensity area in the bilateral basal ganglia and right occipital cortical (Fig. 1B). Magnetic resonance angiography (MRA; TR=25 ms, TE=3.4 ms, FOV=200 mm, matrix size=512*512, slice thickness=1.1mm, gapless, NSA=1, flip angle=20-degree, Scan technique=Inversion recovery) showed segmental narrowing and dilatation, called “sausage of the strings”, in the bilateral middle and posterior cerebral artery (Fig. 1C). Neck MRA showed irregular stenosis of the left vertebral artery (VA) at the level of third-fourth cervical vertebra (Fig. 1D). Neither infarction nor brain aneurysm was detected. In the evaluation of the three-dimensional black blood T1-weighted imaging using the motion-sensitized driven equilibrium 3D turbo spin echo (MSDE; TR=shortest, TE=shortest, FOV=230mm, matrix size=512*512, slice thickness= 0.7mm, gapless, NSA=2, flip angle=90-degree, Scan technique=spin echo) sequence method, coronal (Fig. 1E) and axial view (Fig. 1F) revealed a periluminal rim, called crescent sign, suggested an intramural hematoma (IMH) consistent with the extracranial vertebral artery stenosis area (Fig. 1E, F). We speculated that RCVS, PRES and CAD occurred almost at about the same time.
We treated her with rest and intensive hypotension without antithrombotic therapy. After discharge, her clinical course was good with no progression. After three months, CT and MRI revealed the disappearance of SAH and FLAIR high intensity legion (Fig. 2A). MRA showed improvement of segmental narrowing (Fig. 2B). Cervical MRA revealed normal left VA (Fig. 2C) and MSDE (Fig. 2D, E) showed no abnormal signal. These results indicate that these conditions were reversible, and we have judged these reversible lesions as multiple phenomena of postpartum stroke occurred at the same time.
RCVS, which has been described in the literature since the early 1970’s, is characterized by severe headache (especially called thunderclap headache), with or without other acute neurological symptoms, and diffuse segmental vasoconstriction of cerebral arteries, which is reversible within 3 months.5 It’s common in postpartum women and called postpartum cerebral angiopathy.3.6 Previous studies revealed that 5-12% of RCVS patients are postpartum women.7, 8 As neuroradiorogical findings of RCVS, in one third to a half of cases reveal cSAH, reversible cerebral edema called PRES, cervicocarotid dissection (CAD), intraparenchymal hemorrhage, subdural hemorrhage and ischemic stroke.3, 9-12 cSAH is the most frequent finding of RCVS and was reported to account for 30-38% of hemorrhagic complications.8, 9 cSAH associated with postpartum angiopathy could result from rupture or reperfusion injury affecting the small arteries of the leptomeninges.13 The diagnosis of RCVS should be considered in patients who present with recurrent thunderclap headache, with or without focal neurological signs and non-aneurysmal SAH.14 PRES is one of the hypertensive encephalopathies that can cause cerebral edema, seizures, and both ischemic and hemorrhagic strokes.15 It was reported that approximately 4% of 95 women with postpartum headache were diagnosed with PRES.1 Bartynski et al. reported that the parietal and occipital lobes were the most frequently abnormal regions in 134 patients of PRES, but lesions of basal ganglia, such as our case, were found in 14% of the series.16
It is suggested that RCVS and PRES are interrelated and share pathophysiologic mechanisms such as small vessel endothelial dysfunction, deficiencies in self-regulation, and decreased sympathetic innervation of the posterior circulation.3, 10, 14, 15 In one prospective study of 77 consecutive male and female patients with RCVS, 7 (9.1%) developed PRES.10
CAD is a major cause of cerebral ischemia and hemorrhage in young adult17, but it is a rare complication during the postpartum period. The presence of intramural hematoma (IMH) is the basis of the diagnosis of CAD.18 IMH, which can be identified on MSDE, characteristically has a crescentic shape adjacent to the vessel lumen and often spirals along the length of the artery.18, 19, 20 Previous reports suggested that the MSDE-3D-TSE sequence was useful for assessing vessel walls in the dissection and aneurysms.21-23 MSDE causes phase dispersion of blood spin using a magnetic field to suppress blood flow signal, producing 3D T1-weighted images.24 Digital subtraction angiography (DSA) is the gold standard for diagnosis and follow-up of CAD; however, it is invasive and cannot determine IMH.17 Other angiographic features are luminal flap, false lumen, long tapered stenosis, and dissecting aneurysms.17 We were able to confirm the presence of intramural hematoma and the process of its change, so we have diagnosed with CAD on MRI-MSDE method.
To the best of our knowledge, 26 women and 35 dissections have described postpartum patients with CAD, including our patient (Table 1).25-36 The mean age was 35 years (range, 26-44 years); the mean time from birth to onset was 10.5 days (range, 1 hour-53 days). For CAD-affected lesions, 15 (57%) women had a single lesion and the other women (43%) had multiple lesions. Among the 26 women with CAD, 6 (23%) had SAH, 6 (23%) had RCVS, and 9 (34%) had infarction. One case had PRES based on FLAIR high-intense area in the bilateral occipital area.34 Previous reports indicate that 8-12% of RCVS patients have CAD.13, 37 Wiebers et al. described the first case of a 44-year-old woman with postpartum CAD in 1985.25 She developed infarction in the left MCA territory and was treated with heparin. The first CAD-related RCVS case was diagnosed incidentally.38 Arnold et al. reported that 6% of 102 postpartum women with CAD.30 Interestingly, the prognosis was good with or without antithrombotic therapy.
According to our case and previous reports, we speculate that postpartum vascular lesions are a series of pathological conditions. Although not confirmed in our case, RCVS and CAD are associated with alteration of the vessel wall of the vasa vasorum, vasculopathy-related arterial tears and genetic predisposition.6, 10, 39, 40 RCVS and PRES share a vulnerability in the posterior circulation. 10, 39 These pathologies have been speculated to include small vessel endothelial dysfunction, defective autoregulation, and reduced sympathetic innervation of the posterior circulation.10, 39, 41
In some pathological reports, skin biopsy of CAD patients revealed ultrastructural derangements of type I and type III collagen fibrils. 42, 43 Another study including superficial arterial biopsy of CAD patients showed degradation with inflammatory and erythrocytic accumulation between the tunica media and adventitia. 44
In pregnancy and postpartum periods, endothelial damage associated with sudden changes in blood pressure led to the destruction of the blood-brain barrier. 5, 45 In addition, increased activity of the immune system can activate proteolytic enzymes such as matrix metalloproteinases and cause weakening of the arterial wall. 44 A series of conditions may indicate arterial wall instability and dysfunction. Our case began with stiff shoulders and then became aware of thunderclap headache. From this mode of onset, CAD first occurred, and we speculated that RCVS and PRES could be induced by being aware of stiff shoulders and pain.
Our case was rare in that multiple phenomena of postpartum stroke occurred at the same time. What is remarkable about this case was the confirmation of the reversibility of these pathological conditions through multifaceted evaluations, including the MSDE sequencing method. And these pathologies were the same properties, are reversible, and show a good prognosis with hypotension and rest treatment, without antithrombotic therapy.
Non-financial interest in the subject matter or materials discussed in this manuscript.
This study was conducted according to the principles of the Declaration of Helsinki.
his article does not contain any studies with human participants performed by any of the authors.
Informed consent was obtained from all individual participants .
Due to technical limitations, table 1 is only available as a download in the Supplemental Files section.