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Table of Contents    
Year : 2013  |  Volume : 61  |  Issue : 5  |  Page : 517-522

Postpartum cerebral angiopathy presenting with non-aneurysmal subarachnoid hemorrhage and interval development of neurological deficits: A case report and review of literature

1 Department of Neurology, The Second Xiangya Hospital, Central South University, Changsha, Hunan 410011, P.R. China
2 Department of Radiology, Hospital of the University of Pennsylvania, Philadelphia, PA 19104, USA

Date of Submission03-Sep-2013
Date of Decision05-Sep-2013
Date of Acceptance20-Oct-2013
Date of Web Publication22-Nov-2013

Correspondence Address:
Li Yang
Department of Neurology, The Second Xiangya Hospital, Central South University, No. 139 Middle Renmin Road, Changsha, Hunan - 410011
P.R. China
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Source of Support: This work was supported by the Natural Science Foundation of China (No. 81301988) to Li Yang, Conflict of Interest: None

DOI: 10.4103/0028-3886.121933

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 » Abstract 

Postpartum cerebral angiopathy (PCA) is a cerebrovascular disease that occurs during the postpartum period. It is characterized by reversible multifocal vasoconstriction of the cerebral arteries. We report a patient with PCA proven by cerebral angiography that revealed multifocal, segmental narrowing of the cerebral arteries and non-aneurysmal subarachnoid hemorrhage. The patient suddenly deteriorated with focal neurological deficits on the 5 th day of hospitalization. She was treated with calcium-channel blockers and monitored with daily transcranial Doppler ultrasound. Her symptoms gradually improved and she was discharged on the 11 th day of hospitalization. At 1-month follow-up, patient was completely symptom-free with no neurological deficits.

Keywords: cerebral vasospasm, postpartum cerebral angiopathy, reversible cerebral vasoconstriction syndrome, subarachnoid hemorrhage

How to cite this article:
Yang L, Bai HX, Zhao X, Xiao Y, Tan L. Postpartum cerebral angiopathy presenting with non-aneurysmal subarachnoid hemorrhage and interval development of neurological deficits: A case report and review of literature. Neurol India 2013;61:517-22

How to cite this URL:
Yang L, Bai HX, Zhao X, Xiao Y, Tan L. Postpartum cerebral angiopathy presenting with non-aneurysmal subarachnoid hemorrhage and interval development of neurological deficits: A case report and review of literature. Neurol India [serial online] 2013 [cited 2023 Dec 10];61:517-22. Available from:

 » Introduction Top

Postpartum cerebral angiopathy (PCA) is one of the reversible cerebral vasoconstriction syndromes (RCVS) that occurs within the first 2 weeks postpartum in women with an uneventful pregnancy and delivery. [1] It is characterized by multifocal, diffuse, segmental narrowing of the cerebral arteries on angiography. [2] The clinical features of PCA include recurrent acute severe headaches (thunderclap), nausea, vomiting, photophobia, seizures, hypertension, confusion and focal neurological deficits. [3] PCA is typically self-limited. [4] In some cases, it can be associated with either intracranial hemorrhage or ischemic stroke. Subarachnoid hemorrhage (SAH) is a less common sequela of PCA. [5] Despite its generally benign character, PCA can produce severe neurological deficits, which compel clinicians to initiate prompt and aggressive treatment. [6] Here, we report a case of PCA complicated by non-aneurysmal SAH and interval development of focal neurological deficits.

 » Case Report Top

This paper reports a case of a 30-year-old female patient who presented on post-partum day-10 with hypertension, headache, nausea/vomiting and photophobia. Patient first developed severe thunderclap headache on post-partum day-5. The headache was throbbing and not relieved by over-the-counter pain medications. It was accompanied by photophobia and nausea/vomiting. She presented to the emergency room on post-partum day-7. After a negative head computed tomography (CT), she was given fioricet for possible migraine and sent home. On post-partum day-10, patient returned to the emergency room with hypertension to the 180s systolic, headache, nausea/vomiting, and photophobia. Neurological exam was non-focal. She was sent to labor and delivery due to concern for preeclampsia. An intravenous infusion of magnesium was started empirically to treat postnatal preeclampsia. Her blood pressure improved, but her headache was unresponsive to narcotics. Patient is G3P4 with no preeclampsia or other issues with pregnancy. Past medical history includes migraine headache. Patient smokes one pack of cigarettes per day, but denies alcohol or illicit drugs.

Magnetic resonance imaging, MR-venography was negative for infarct or thrombosis. However, there was right frontal lobe cortical SAH. CT revealed right SAH involving the right anterior suprasellar cistern coursing laterally and extending into the right sylvian fissure [Figure 1]. Patient was started on keppra, labetolol and nimodipine. CT angiography (CTA) of the head and circle of Willis was unremarkable with no evidence of aneurysm. Diagnostic angiogram showed multifocal, circumferential narrowing of multiple vessels within the anterior circulation, bilateral PCA and left posterior inferior cerebellar artery (PICA) [Figure 2]. Levetericetam was discontinued and nimodipine was switched to verapamil. Patient's headache improved. Lumbar puncture and erythrocyte sedimentation rate/C-reactive protein were not performed since the suspicion for vasculitis was low.
Figure 1: Diffusion-weighted magnetic resonance imaging on postpartum day 10 showed increased signal in the anterolateral right frontal sulci suggesting subarachnoid hemorrhage. (b) Non-contrast computed tomography (CT) demonstrated subarachnoid hemorrhage, which appears to extend from the right anterolateral aspect of the suprasellar cistern along the right sylvian fissue. (c) Enhanced CT showed enhancement of hyperdense material noted within the anterolateral right frontal sulci

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Figure 2: Cerebral angiography showed (a) multifocal, circumferential narrowing involving the anterior circulation including the middle cerebral artery (MCA) from M1 to distal M4 branches, as well as the anterior cerebral artery (ACA) from the A1 segment to distal A4 branches. (b) Multifocal, circumferential narrowing involving the anterior circulation including distal MCA branches, as well as the ACA from the A1 segment to distal A4 branches. (c) 3D reconstruction of computed tomography angiogram demonstrated multifocal, segmental constriction of the cerebral arteries

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On the 5 th day of hospitalization (postpartum day-15), the patient suddenly developed left-sided arm weakness and facial droop. CT demonstrated interval development of small transcortical infarct in the right frontal lobe [Figure 1]. CT angiogram revealed progressing segmental arterial narrowing in the anterior and posterior circulations [Figure 2]. Worsening of her neurological status was associated with increased transcranial Doppler (TCD) velocities [Figure 3]. Her headache subsequently improved over the few days and the neurological symptoms resolved. A repeat cerebral angiogram was not performed. Patient was discharged from the hospital on day 11. Her final diagnosis was PCA with SAH. At 1-month follow-up, patient was well with no recurrence of symptoms.
Figure 3: Mean velocities of right and left anterior cerebral artery and middle cerebral artery segments on transcranial Doppler sonography with respect to postpartum day. *Neurological deterioration happened on postpartum day 15

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 » Discussion Top

PCA is a well-recognized subgroup of RCVS. [1],[4] It is typically self-limited and occurs within the 1 st week postpartum in women after an uneventful pregnancy and delivery. [4] Cerebral angiography depicts multifocal, diffuse, segmental narrowing of cerebral arteries that resolve within weeks of presentation. [2] The clinical features can include severe headache (usually characterized as "thunderclap" with an acute and excruciating onset), nausea, vomiting, photophobia, seizures, hypertension, confusion and focal neurological signs. [3] Sequelae of PCA commonly includes both ischemic strokes and intracerebral hemorrhages. [7],[8] SAH is rare in the context of PCA. [5] Rapid neurological deterioration is a known complication after the initial presentation. [2],[9],[10],[11],[12],[13] The clinical course of PCA is typically benign, although neurological impairment (and even death) can result from progressive vasoconstriction, stroke and brain edema. [7],[14],[15] To the best of our knowledge, only two cases of PCA patients presenting with both SAH and interval development of neurological deficits have been previously reported. [16],[17]

In our case, diagnosis was narrowed to PCA and central nervous system (CNS) vasculitis on the basis of angiography. PCA is difficult to differentiate from CNS vasculitis by angiographic findings alone. However, clinical findings that include rapid improvement of symptoms and reversible vasoconstriction excluded the diagnosis of vasculitis. An aneurysm was not detected on magnetic resonance angiography (MRA)/CTA or angiography. In addition, the time course of vasospasm onset is atypical for vasospasm related to aneurismal SAH. [18] Patient did not show any evidence for eclampsia, including hypertension, proteinuria, edema or toxemia. Clinical findings such as thunderclap headache, typical angiographic findings and rapid clinical improvement, as well as a temporal relationship to pregnancy lead to the diagnosis of PCA in our patient. Consequently, aggressive treatment was not pursued even when the patient experienced neurological deficits on day 5.

A total of 13 cases of imaging-proven PCA have been reported previously [Table 1]. The median age was 31.5. Cerebral vasoconstriction was associated with SAH in seven cases [5],[9],[13],[16],[17],[19] and with intracranial hemorrhage in nine. [7],[10],[12],[20],[21],[22],[23] None of the patients who presented with SAH had an aneurysm identified as the cause. The majority of patients presented within 2 weeks postpartum. Five occurred immediately after delivery, [5],[9],[16],[24],[25] while one had a delayed onset at 1 month. [10] At presentation, acute hypertension was detected in 11 cases. [5],[10],[11],[15],[16],[19],[21],[22],[23],[24],[26] 15 experienced were generalized tonic-clonic seizures a few days after presentation. [5],[6],[9],[10],[11],[12],[15],[22],[26],[27],[28],[29] Seven were linked to the administration of vasospastic drug. [12],[23],[26],[27],[28],[29],[30] Eight mentioned a history of migraines. [5],[7],[9],[11],[20],[22],[28],[29] Segmental vasoconstriction was demonstrated on cerebral angiography in 25 cases, MRA in 8, [6],[7],[11],[15],[17],[19],[31],[32] and CTA in 2. [7],[15] Fourteen had elevated blood flow velocities on TCD. [5],[6],[9],[10],[13],[14],[15],[17],[19],[22],[24],[25],[29],[32] Sixteen suffered deterioration with focal neurological deficits. [2],[6],[7],[10],[11],[12],[14],[15],[16],[17],[20],[22],[23],[25],[30] 18 patients were treated with steroids, [6],[7],[10],[12],[13],[14],[15],[16],[17],[20],[21],[22],[23],[25],[28],[30] sixteen with calcium-channel blockers, [5],[6],[7],[9],[14],[15],[16],[17],[19],[21],[22],[23],[27],[30],[31] and eight with intravenous magnesium, [5],[6],[9],[10],[11],[17],[22],[30] three with cyclophosphamide, [10],[22],[30] three with balloon angioplasty, [6],[7] two with beta-blockers, [16],[19] and one with IVIg. [17] The median follow-up was 2 months. 23 reported resolution or significant improvement of headache; four were left with a neurological deficit; [10],[20],[21],[31] five patient died at a median of 12 days after presentation. [7],[14],[15],[22] Reversibility of the segmental vasoconstriction was confirmed by follow-up TCD in 13 patients, [5],[6],[9],[10],[13],[17],[19],[24],[25],[26],[29],[32] MRA in 6, [2],[10],[11],[27],[31],[32] and angiography in 5. [5],[12],[16],[20],[28] Overall, PCA patients presenting with SAH does not seem to have a worse outcome than those without. On the other hand, those with intracranial hemorrhage and infarction were associated with a worse outcome. [7],[8]
Table 1: Characteristics of studies of postpartum cerebral angiopathy

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The pathogenesis of PCA is poorly understood. It has been suggested that postpartum preclampsia, eclampsia and PCA are within the same spectrum of peripartum cerebrovascular illness and share a common pathophysiological process. [9] Pregnancy-related hormonal changed seem to induce remodeling of the arterial walls during the period of pregnancy. [33] The transient vasospasm could be either the cause of or response to acute severe hypertension. Furthermore, an unknown genetic pre-disposition might lead to hypersensitivity reactions to the postpartum physiology or hypertension, inducing vasoconstriction through the alpha-adrenergic receptors distributed on cerebral vessels. [21] Brain autopsy findings of women with PCA have shown marked inflammatory cell infiltrate of intraparenchymal arterioles [22] and slight intimal proliferation. [14]

 » Conclusion Top

Our case report underscores the importance of considering PCA in the differential diagnosis of postpartum patients presenting with thunderclap headaches and non-aneurysmal SAH overlying the cortical surfaces. Acute worsening with neurological deficits is common. A benign outcome can be expected despite significant intracranial arterial vasoconstriction. Clinicians should refrain from pursuing unnecessarily aggressive and potentially toxic treatment when the diagnosis is certain. TCD can be useful for monitoring the disease course in this condition.

 » Acknowledgement Top

This work was supported by the Natural Science Foundation of China (No. 81301988) to Li Yang.

 » References Top

1.Ducros A, Bousser MG. Reversible cerebral vasoconstriction syndrome. Pract Neurol 2009;9:256-67.  Back to cited text no. 1
2.Chandrashekaran S, Parikh S, Kapoor P, Subbarayan S. Postpartum reversible cerebral vasoconstriction syndrome. Am J Med Sci 2007;334:222-4.  Back to cited text no. 2
3.Calado S, Viana-Baptista M. Benign cerebral angiopathy; postpartum cerebral angiopathy: Characteristics and treatment. Curr Treat Options Cardiovasc Med 2006;8:201-12.  Back to cited text no. 3
4.Ducros A, Boukobza M, Porcher R, Sarov M, Valade D, Bousser MG. The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome. A prospective series of 67 patients. Brain 2007;130:3091-101.  Back to cited text no. 4
5.Edlow BL, Kasner SE, Hurst RW, Weigele JB, Levine JM. Reversible cerebral vasoconstriction syndrome associated with subarachnoid hemorrhage. Neurocrit Care 2007;7:203-10.  Back to cited text no. 5
6.Song JK, Fisher S, Seifert TD, Cacayorin ED, Alexandrov AV, Malkoff MD, et al. Postpartum cerebral angiopathy: Atypical features and treatment with intracranial balloon angioplasty. Neuroradiology 2004;46:1022-6.  Back to cited text no. 6
7.Fugate JE, Ameriso SF, Ortiz G, Schottlaender LV, Wijdicks EF, Flemming KD, et al. Variable presentations of postpartum angiopathy. Stroke 2012;43:670-6.  Back to cited text no. 7
8.Ducros A, Fiedler U, Porcher R, Boukobza M, Stapf C, Bousser MG. Hemorrhagic manifestations of reversible cerebral vasoconstriction syndrome: Frequency, features, and risk factors. Stroke 2010;41:2505-11.  Back to cited text no. 8
9.Chik Y, Hoesch RE, Lazaridis C, Weisman CJ, Llinas RH. A case of postpartum cerebral angiopathy with subarachnoid hemorrhage. Nat Rev Neurol 2009;5:512-6.  Back to cited text no. 9
10.Geocadin RG, Razumovsky AY, Wityk RJ, Bhardwaj A, Ulatowski JA. Intracerebral hemorrhage and postpartum cerebral vasculopathy. J Neurol Sci 2002;205:29-34.  Back to cited text no. 10
11.Ghia D, Cuganesan R, Cappelen-Smith C. Delayed angiographic changes in postpartum cerebral angiopathy. J Clin Neurosci 2011;18:435-6.  Back to cited text no. 11
12.Roh JK, Park KS. Postpartum cerebral angiopathy with intracerebral hemorrhage in a patient receiving lisuride. Neurology 1998;50:1152-4.  Back to cited text no. 12
13.Ursell MR, Marras CL, Farb R, Rowed DW, Black SE, Perry JR. Recurrent intracranial hemorrhage due to postpartum cerebral angiopathy: Implications for management. Stroke 1998;29:1995-8.  Back to cited text no. 13
14.Geraghty JJ, Hoch DB, Robert ME, Vinters HV. Fatal puerperal cerebral vasospasm and stroke in a young woman. Neurology 1991;41:1145-7.  Back to cited text no. 14
15.Singhal AB, Kimberly WT, Schaefer PW, Hedley-Whyte ET. Case records of the Massachusetts General Hospital. Case 8-2009. A 36-year-old woman with headache, hypertension, and seizure 2 weeks post partum. N Engl J Med 2009;360:1126-37.  Back to cited text no. 15
16.Lee SY, Sheen SH, Lee SH, Kim SS, Kim CH, Yie KS, et al. Postpartum cerebral angiopathy presenting with non-aneurysmal subarachnoid hemorrhage. J Clin Neurosci 2011;18:1269-71.  Back to cited text no. 16
17.Samaniego EA, Dabus G, Generoso GM, Tari-Capone F, Fuentes K, Linfante I. Postpartum cerebral angiopathy treated with intra-arterial nicardipine and intravenous immunoglobulin. J Neurointerv Surg 2013;5:e12.  Back to cited text no. 17
18.Saito I, Ueda Y, Sano K. Significance of vasospasm in the treatment of ruptured intracranial aneurysms. J Neurosurg 1977;47:412-29.  Back to cited text no. 18
19.Singhal AB, Bernstein RA. Postpartum angiopathy and other cerebral vasoconstriction syndromes. Neurocrit Care 2005;3:91-7.  Back to cited text no. 19
20.Modi M, Modi G. Case reports: Postpartum cerebral angiopathy in a patient with chronic migraine with aura. Headache 2000;40:677-81.  Back to cited text no. 20
21.Thakur JD, Chittiboina P, Khan IS, Nanda A. Unique case of postpartum cerebral angiopathy requiring surgical intervention: Case report and review of literature. Neurol India 2011;59:891-4.  Back to cited text no. 21
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22.Williams TL, Lukovits TG, Harris BT, Harker Rhodes C. A fatal case of postpartum cerebral angiopathy with literature review. Arch Gynecol Obstet 2007;275:67-77.  Back to cited text no. 22
23.Comabella M, Alvarez-Sabin J, Rovira A, Codina A. Bromocriptine and postpartum cerebral angiopathy: A causal relationship? Neurology 1996;46:1754-6.  Back to cited text no. 23
24.Cho AH, Lim SC, Kim BS, Yang DW, Shon YM. Atypical presentation of postpartum cerebral angiopathy shown as a small penetrating arterial territory infarct with severe peri-infarct edema. J Neuroimaging 2010;20:290-1.  Back to cited text no. 24
25.Kubo S, Nakata H, Tatsumi T, Yoshimine T. Headache associated with postpartum cerebral angiopathy: Monitoring with transcranial color-coded sonography. Headache 2002;42:297-300.  Back to cited text no. 25
26.Bogousslavsky J, Despland PA, Regli F, Dubuis PY. Postpartum cerebral angiopathy: Reversible vasoconstriction assessed by transcranial Doppler ultrasounds. Eur Neurol 1989;29:102-5.  Back to cited text no. 26
27.Granier I, Garcia E, Geissler A, Boespflug MD, Durand-Gasselin J. Postpartum cerebral angiopathy associated with the administration of sumatriptan and dihydroergotamine - A case report. Intensive Care Med 1999;25:532-4.  Back to cited text no. 27
28.Janssens E, Hommel M, Mounier-Vehier F, Leclerc X, Guerin du Masgenet B, Leys D. Postpartum cerebral angiopathy possibly due to bromocriptine therapy. Stroke 1995;26:128-30.  Back to cited text no. 28
29.Raroque HG Jr, Tesfa G, Purdy P. Postpartum cerebral angiopathy. Is there a role for sympathomimetic drugs? Stroke 1993;24:2108-10.  Back to cited text no. 29
30.Konstantinopoulos PA, Mousa S, Khairallah R, Mtanos G. Postpartum cerebral angiopathy: An important diagnostic consideration in the postpartum period. Am J Obstet Gynecol 2004;191:375-7.  Back to cited text no. 30
31.Akins PT, Levy KJ, Cross AH, Goldberg MP, Schieber MH. Postpartum cerebral vasospasm treated with hypervolemic therapy. Am J Obstet Gynecol 1996;175:1386-8.  Back to cited text no. 31
32.Neudecker S, Stock K, Krasnianski M. Call-Fleming postpartum angiopathy in the puerperium: A reversible cerebral vasoconstriction syndrome. Obstet Gynecol 2006;107:446-9.  Back to cited text no. 32
33.Akhter T, Larsson A, Larsson M, Wikström AK, Naessen T. Artery wall layer dimensions during normal pregnancy: A longitudinal study using noninvasive high-frequency ultrasound. Am J Physiol Heart Circ Physiol 2013;304:H229-34.  Back to cited text no. 33


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