Article Text
Abstract
We describe two women presenting with severe postpartum headache associated with hypertension but with no other signs or investigation results to suggest pre-eclampsia. In one case, the headache was associated with atypical subarachnoid haemorrhage. The variable nature of the headache and the degree of associated hypertension raised the clinical suspicion of reversible cerebral vasoconstriction syndrome, confirmed on MR angiography. Both patients took nimodipine until the cerebral vasoconstriction had resolved radiologically.
- HEADACHE
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Case reports
Case 1
A 37-year-old woman presented with a severe band-like headache, 36 days after an uncomplicated pregnancy and emergency caesarean delivery at term for fetal distress. She had not been given ergot alkaloid drugs. Her blood pressure was 184/111 mm Hg. Her initial management was with opiate analgesia, intravenous magnesium and intravenous labetalol; however, the intermittent severe headaches continued, always associated with a rise in blood pressure. At the onset of headache and hypertension, she would become confused and disorientated for a few minutes, being unaware that she was in hospital or had delivered a baby; this would then spontaneously resolve. There was no history of antenatal hypertension or of migraine. Her examination was otherwise normal with no neck stiffness or focal neurological signs. Urinalysis showed no proteinuria. Owing to her confusion, we treated her empirically with aciclovir. CT scan of brain showed no evidence of intra-axial or extra-axial haemorrhage and no venous sinus thrombosis. Her lumbar puncture opening pressure was 19 cm H2O and her cerebrospinal fluid (CSF) was sterile. Because of her variable symptoms in association with intermittent hypertension, we arranged MR angiography, which showed bilateral beading of the anterior cerebral arteries consistent with cerebral vasoconstriction (figure 1). After starting nimodipine 60 mg 4 hourly, her blood pressure normalised; her symptoms gradually improved over the next 12 weeks. Repeat MR angiography 3 months later showed no residual vascular narrowing (figure 1). In retrospect, she recalled a headache of similar severity and duration after a previous pregnancy.
Case 2
A 33-year-old woman presented with a sudden-onset severe headache 7 days after a spontaneous vaginal delivery of a well-grown infant. This was her third pregnancy. She had not undergone epidural anaesthesia or been given ergot alkaloids. She described the headache as being more painful than childbirth and ‘tearing’ across her forehead. Although she had experienced migraine since childhood, she was very clear that this was different from her previous temporal migrainous headaches. Her blood pressure was 160/90 mm Hg, and neurological examination was normal. Her routine blood tests were normal, and urinalysis showed no proteinuria. The headache severity was variable during the first 48 h after admission, with recurrent episodes of maximum pain. CT scan of brain showed a 9×7 mm area of increased density within the cortical sulci, suggesting a bleed. MR scan of brain confirmed an atypical subarachnoid bleed and showed bilateral multifocal segmental narrowing of the middle cerebral arteries (figure 2). We prescribed nimodipine 60 mg 4 hourly, and her headache improved over the next 4 weeks. Repeat imaging 1 month later showed resolution of the vasoconstriction (figure 2).
Comment
Postpartum headache occurs in up to 40% of women in the first week after delivery; migrainous and tension-type headaches are the most common. However, in these two cases, the presenting headache was unusually severe and so we investigated them urgently for a secondary cause. Reversible cerebral vasoconstriction syndrome (RCVS) presents with explosive-onset, ‘worst-ever’ headaches, with characteristic multifocal vasoconstriction and vasodilatation of the intracranial arteries.1 Cerebral imaging in both cases was diagnostic for RCVS, with segmental narrowing of the cerebral arteries on MR angiography. In addition, one-third of RCVS cases have convexity subarachnoid haemorrhage with no aneurysm,1 as in the second case. RCVS is considered a rare cause of headache, though its exact incidence and prevalence are unknown.
The pathophysiology of RCVS is not fully understood. CSF, serology, brain biopsy and temporal artery biopsy are typically normal. However, it is more common in women and 10% of cases present postpartum.1 ,2 This epidemiological link to the postpartum period remains unexplained. Although 50–70% of postpartum cases are associated with the use of vasoconstrictive drugs,2 neither of these women had received ergot alkaloid uterotonics. Other physiological or hormonal factors must therefore contribute to its female predominance and postpartum risk.
Patients with RCVS may develop transient hypertension at the time of the headache.1 ,2 However, pre-eclampsia is still the most likely explanation for postpartum headache with hypertension, even without proteinuria. Indeed, postpartum pre-eclampsia is more often associated with symptoms (especially headache) than antenatal pre-eclampsia. As well as headache, pre-eclampsia gives right upper quadrant abdominal pain, oedema and visual changes such as scintillating scotomas; the signs include right upper quadrant tenderness and increased deep tendon reflexes. The abnormal blood tests of severe pre-eclampsia include raised hepatic transaminases, thrombocytopenia and evidence of acute kidney injury. Neither case here showed these. Furthermore, the normal blood pressure at the start of pregnancy (diastolic <80 mm Hg in the first trimester) and the absence of pregnancy-induced hypertension at any stage—either in the pregnancy or in the initial peripartum period—made postpartum pre-eclampsia unlikely. A postpartum patient presenting with hypertension and headache should be considered to have pre-eclampsia until a definite diagnosis is possible. The magnesium sulfate used to manage severe pre-eclampsia acts as a cerebral vasodilator and so may, in theory, help patients with RCVS.
Another differential diagnosis is posterior reversible encephalopathy syndrome (PRES), which shares clinical and radiological features with RCVS and can coexist with it. PRES presents with headache, encephalopathy, seizures and visual disturbance. However, PRES headache is rarely thunderclap. PRES symptoms typically develop over several hours, peak at 24–48 h and seldom last >7 days. This contrasts with RCVS, where severe abrupt headache is a primary feature and it can recur over several weeks.2 Furthermore, the prominent visual disturbances of PRES, including cortical blindness, hemianopia and visual blurring, are uncommon in RCVS. PRES, like RCVS, nearly always shows cerebral vasoconstriction on angiography;2 convexity subarachnoid haemorrhage, intracerebral haemorrhage and cerebral infarction also may complicate either condition.3 However, PRES differs from RCVS in showing vasogenic oedema on MRI (fluid-attenuated inversion recovery sequence), typically manifesting as bilateral, parieto-occipital white matter lesions. PRES has many recognised triggers, including pre-eclampsia, immunosuppressant treatment, renal failure, autoimmune disease and infection. It is unclear whether abrupt hypertension can trigger PRES or whether the acutely elevated blood pressure seen in most patients with PRES is reactive and necessary to maintain cerebral blood flow. Clinicians should consider pre-eclampsia as the trigger to postpartum PRES, unless proven otherwise. Its management includes magnesium sulfate for seizure prophylaxis and antihypertensive treatment. Patients can be reassured that the clinical and radiological features of PRES should resolve as the trigger for eclampsia disappears postpartum.
Both these cases of postpartum RCVS showed variable symptoms and signs. The first case showed waxing and waning of headache, hypertension and cognitive impairment. The second case had a severe headache but with recurrences of its thunderclap element. This variability, together with the atypical nature of the headache, made RCVS more likely in both cases and led to us requesting angiography.
We managed both patients empirically with nimodipine, a cerebrovascular vasodilator. With no data from relevant randomised trials, this was based on expert opinion and reported case series data. However, it is difficult to assess nimodipine's therapeutic benefit given the variable and self-limiting natural history of the disease. In addition, we do not know the optimum duration of treatment. Both women were breast feeding, and we advised them to continue this while taking nimodipine. There are good safety data for other calcium channel blockers in breast feeding, and there is a reported isolated case of a lactating mother taking nimodipine 60 mg 4 hourly, where the baby had negligible exposure (0.0009% of the maternal weight-adjusted dose).4
Our first patient later recalled a headache of similar severity and duration after her first pregnancy: RCVS was possible then, but we have no angiographic imaging from that pregnancy. There are no data regarding the recurrence of postpartum RCVS in subsequent pregnancies, and we could not counsel either patient regarding this. We simply provided written clinical information to help their doctors target relevant investigations and management should this recur after future pregnancies.
Footnotes
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Contributors All the authors contributed to the concept and design of the case report, were involved in drafting and revising the work and in approving the final version for publication.
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Funding KW is funded by a National Institute for Health Research rare disease translational research collaboration fellowship award.
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Competing interests None.
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Patient consent Obtained.
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Provenance and peer review Not commissioned; externally peer reviewed. This paper was reviewed by Nicola Giffin, Bath, UK.
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