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 » Introduction
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Table of Contents    
CASE REPORT
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 2019 Nov 20];61:517-22. Available from: http://www.neurologyindia.com/text.asp?2013/61/5/517/121933



 » 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

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