Reversible cerebral vasoconstriction syndrome following intracranial hypotension in a postpartum patient: a case report and literature review

Abstract

Introduction
Reversible cerebral vasoconstriction syndrome (RCVS) is a potentially life-threatening neurological disorder, rarely linked to intracranial hypotension. The presentation showed a patient with intracranial hypotension after peridural anesthesia who experienced RCVS during the early postpartum period, suggesting a potential involvement of intracranial hypotension in RCVS occurrence.

Case report
A young female of 29 years of age initially developed an orthostatic headache after undergoing a painless delivery with lumbar epidural anesthesia. Intracranial hypotension was considered the underlying cause. Her headache was partially resolved after intravenous fluid therapy and strict bed rest. After 2 days, the patient had a new onset thunderclap headache with generalized seizures, cortical blindness, and elevated blood pressure. An MRI scan revealed high signal intensity within the temporal, parietal, and occipital lobes, left caudate nucleus, and right cerebellum on T2-FLAIR imaging with vasogenic edema. MR angiography indicated multifocal, segmental, diffuse narrowing affecting the cerebral arteries that are large and medium. An RCVS2 score was six, and the patient was diagnosed with RCVS. She was managed conservatively, quickly improving her symptoms. After 10 days, a follow-up MRI indicated a significant reduction in the abnormal signal, and a substantial resolution of the constriction of the cerebral artery constriction was confirmed by MR angiography.

Conclusion
Intracranial hypotension could potentially lead to RCVS in postpartum patients, and it may be triggered by cerebral vasospasm secondary to intracranial hypotension.

Full text

Frontiers in Neurology 01 frontiersin.org
Reversible cerebral
vasoconstriction syndrome
following intracranial hypotension
in a postpartum patient: a case
report and literature review
ShuhuaLi
1†, YiYang
2,3†, JiacaiZuo
2, NingliDu
1 and GuoxianKou
1
*
1 Department of Infectious Diseases, Mianyang Central Hospital, University of Electronic Science and
Technology of China, Mianyang, China, 2 Department of Neurology, Mianyang Central Hospital,
University of Electronic Science and Technology of China, Mianyang, China, 3 Department of Neurology,
West China Hospital, Sichuan University, Chengdu, China
Introduction: Reversible cerebral vasoconstriction syndrome (RCVS) is a potentially
life-threatening neurological disorder, rarely linked to intracranial hypotension.
The presentation showed a patient with intracranial hypotension after peridural
anesthesia who experienced RCVS during the early postpartum period, suggesting
a potential involvement of intracranial hypotension in RCVS occurrence.
Case report: A young female of 29 years of age initially developed an orthostatic
headache after undergoing a painless delivery with lumbar epidural anesthesia.
Intracranial hypotension was considered the underlying cause. Her headache
was partially resolved after intravenous fluid therapy and strict bed rest. After
2 days, the patient had a new onset thunderclap headache with generalized
seizures, cortical blindness, and elevated blood pressure. An MRI scan revealed
high signal intensity within the temporal, parietal, and occipital lobes, left caudate
nucleus, and right cerebellum on T2-FLAIR imaging with vasogenic edema. MR
angiography indicated multifocal, segmental, diuse narrowing aecting the
cerebral arteries that are large and medium. An RCVS2 score was six, and the
patient was diagnosed with RCVS. She was managed conservatively, quickly
improving her symptoms. After 10 days, a follow-up MRI indicated a significant
reduction in the abnormal signal, and a substantial resolution of the constriction
of the cerebral artery constriction was confirmed by MR angiography.
Conclusion: Intracranial hypotension could potentially lead to RCVS in postpartum
patients, and it may betriggered by cerebral vasospasm secondary to intracranial
hypotension.
KEYWORDS
intracranial hypotension, RCVS, cerebral vasospasm, postpartum, case report
Introduction
Reversible cerebral vasoconstriction syndrome (RCVS) is a potentially fatal and uncommon
disease (1). It is usually benign following a monophasic and self-limited process. However, some
patients could suer from catastrophic forms, including ischemic stroke, intracranial
hemorrhage, cortical subarachnoid hemorrhage, posterior reversible encephalopathy syndrome
OPEN ACCESS
EDITED BY
Sait Ashina,
Beth Israel Deaconess Medical Center,
Harvard Medical School, UnitedStates
REVIEWED BY
Thomas Berk,
Thomas Jeerson University Hospital,
UnitedStates
Shazia Afridi,
Guy’s and St Thomas’ NHS Foundation Trust,
UnitedKingdom
*CORRESPONDENCE
Guoxian Kou
854506708@qq.com
†These authors have contributed equally to this
work
RECEIVED 21 August 2023
ACCEPTED 21 September 2023
PUBLISHED 12 October 2023
CITATION
Li S, Yang Y, Zuo J, Du N and Kou G (2023)
Reversible cerebral vasoconstriction syndrome
following intracranial hypotension in a
postpartum patient: a case report and literature
review.
Front. Neurol. 14:1281074.
doi: 10.3389/fneur.2023.1281074
COPYRIGHT
© 2023 Li, Yang, Zuo, Du and Kou. This is an
open-access article distributed under the terms
of the Creative Commons Attribution License
(CC BY). The use, distribution or reproduction
in other forums is permitted, provided the
original author(s) and the copyright owner(s)
are credited and that the original publication in
this journal is cited, in accordance with
accepted academic practice. No use,
distribution or reproduction is permitted which
does not comply with these terms.
TYPE Case Report
PUBLISHED 12 October 2023
DOI 10.3389/fneur.2023.1281074

Li et al. 10.3389/fneur.2023.1281074
Frontiers in Neurology 02 frontiersin.org
(PRES), and death (2, 3). Small distal arteries are rst aected by this
vasospastic disease causing thunderclap headaches, hemorrhagic
strokes, and PRES. Later, the disorder leads to ischemic strokes
involving the medium and large arteries (4). RCVS has an unknown
pathophysiological process, but endothelial dysfunction, sympathetic
hyperstimulation, and alteration of vascular smooth muscle by
oxidative stress have been postulated (5).
Intracranial hypotension is mainly presenting with an orthostatic
headache accompanied by low opening pressure of cerebrospinal uid
(CSF) (<60 mmH20). Intracranial hypotension aer the leakage of
CSF may mechanically stimulate the arterial wall due to displacement
of the brain anatomy. Moreover, the adrenergic system is activated,
leading to cerebral vessel vasospasm and RCVS occurrence (6–8).
Multiple potential etiologies of RCVS have been previously identied,
but the association between intracranial hypotension and RCVS is
rarely discussed. Limited reports have proposed RCVS may trigger by
intracranial hypotension, and the pathophysiology is not clearly
known at this time (6). Here, a postpartum patient was presented with
intracranial hypotension aer peridural anesthesia with
subsequent RCVS.
Case report
A young female who was 29 years old presented to the obstetrical
department and underwent a painless delivery with lumbar epidural
anesthesia at 39 weeks +6/7 gestation. Aer 8 hours of giving birth, the
patient experienced an orthostatic headache. e cause was suspected
of intracranial hypotension caused by CSF leakage due to an
inadvertent epidural puncture. Aer receiving intravenous uid
therapy and strict bed rest, her symptoms were partially resolved in
the next 2 days. However, the patient developed a new-onset
thunderclap headache that peaked in less than a minute, lasting for
more than 5 minutes, and diering from the previous headaches. She
also reported blurred vision and recurrent generalized tonic–clonic
seizures, requiring an emergency consult from a neurologist. She had
vital signs with a blood pressure of 170/110 mmHg, a pulse rate of 92
beats/min, and a body temperature of 36.9°C. e neurological
examination indicated cortical blindness, which was otherwise
unremarkable. e complete blood counts, inammatory markers,
and standard chemistry panels were normal. e patient had a
new-onset thunderclap headache with epileptic seizures and cortical
blindness, suggesting a lesion in the cerebral cortex. An MRI scan
revealed hyperintensities inside the temporal, parietal, and occipital
lobes, le caudate nucleus, and right cerebellum on T2-FLAIR
imaging (Figure1A) without the restriction of diusion displayed on
diusion-weighted imaging. Postpartum, the patient had an acute
onset of symptoms with multiple brain lesions, indicating
inammatory or vascular disorders as the probable causes. Even
though the intracranial infection could have been a possible cause, the
patient had no fever, with normal inammatory markers and negative
meningeal signs. Additionally, the widespread enhancement of the
dura mater indicated by the enhanced MRI could have been caused
by low intracranial pressure syndrome (Figure2), further decreasing
the intracranial infection possibility. Cerebral vascular examinations
were conducted to clarify the cause of the disease. Due to the
peripartum status of the patient, cerebral venous sinus thrombosis was
considered a possible cause. However, MR venography gave a normal
result, leading to the exclusion of cerebral venous infarction. MR
angiography indicated multifocal, diuse, and segmental narrowing
of arteries (both large and medium) within the anterior and posterior
circulations, with occasional dilation of the segments (Figure1B). e
patient had an RCVS
2
score of six (Figure3). e clinical and imaging
features were consistent with RCVS, and abnormal hypersignals on
MRI FLAIR indicated vasogenic edema, depicting PRES. e patient
was conservatively managed with continued bed rest and intravenous
uid infusion with a daily dosage of 2000 mL for 9 days. Moreover, a
calcium channel antagonist (nifedipine 10 mg qd) and intravenous
10 mg diazepam followed by an antiepileptic drug (levetiracetam, 0.5 g
bid) were also administered. ese interventions quickly resolved her
headache symptoms with vision recovery and no further seizures.
Aer 10 days, another MRI was performed, which indicated markedly
attenuated abnormal signals (Figure 1C). MRA showed that the
cerebral arteries, which were diuse and segmentally constricted, were
improved signicantly (Figure1D).
Discussion
RCVS is a neurovascular condition with characteristics of
thunderclap headache which may or may not beassociated with
further neurological deficits that are focally placed. Additionally,
RCVS is accompanied by seizures and diffuse segmental cerebral
artery narrowing, improving within 3 months of onset (1).
Approximately 70% of RCVS patients possess a known
precipitating factor, with postpartum state, vasoactive drugs, and
nasal decongestants being the primary recognized triggers (5, 9).
A recent study demonstrated that the RCVS prevalence rate
(11.9%) is relatively high among postpartum patients (10).
However, a prospective study investigating 900 consecutive
puerperae observed that the risk of puerperal RCVS is negligible,
with only a 0.1% proportion (11). Therefore, intracranial
hypotension may becrucial in RCVS pathogenesis other than the
potential postpartum trigger.
Currently, the potential mechanisms concerning RCVS
secondary to intracranial hypotension remain speculative. Chaves
etal. (7) described a patient with diuse cerebral vasospasm in
intracranial hypotension and explained that severe CSF volume
reduction could have triggered the vasospasm. Schievink etal. (12)
presented a case with intracranial hypotension with severe,
transient, and segmental cerebral arterial stenosis. ey believed
intracranial hypotension could bethe underlying cause of reversible
cerebral vasoconstriction. Caranzano et al. (13) indicated that
intracranial hypotension could have been the primum movens of
RCVS and cerebral venous sinus thrombosis. Cerebral vasospasm
among patients with intracranial hypotension may betriggered by
stimulating the arterial wall with mechanical force, which occurs by
displacing the brain anatomy through CSF quantity reduction (7,
8). According to the doctrine provided by Monro-Kellie, the brain,
CSF, and blood volume inside the cranium remain constant. CSF
leakage leads to a decrease in intracranial volume. us, the
intracranial venous system may bedilated as compensation, leading
to adrenergic overstimulation and subsequent cerebral vessel
vasospasm (6).
In a postpartum patient, positional headaches following
epidural anesthesia and the presence of neurological signs guide

Li et al. 10.3389/fneur.2023.1281074
Frontiers in Neurology 03 frontiersin.org
the diagnosis of post-dural puncture headache (PDPH), occurring
from intracranial hypotension due to leakage of CSF inside the
epidural space through the location of dural puncture (14). PDPH
may lead to neurological complications, such as persistent
headache, subdural hematoma, depression, cerebral venous
thrombosis, or bacterial meningitis (15). However, RCVS
secondary to intracranial hypotension has limited reports.
We suspected RCVS in our patient when the clinical headache
features changed from positional to thunderclap headache (TCH)
postpartum. RCVS is a common cause of TCH, except for
subarachnoid hemorrhage (SAH), ischaemic stroke, cerebral
venous sinus thrombosis, cervical artery dissection, intracranial
infection and pituitary apoplexy (16). Moreover, RCVS can
manifest as neurological symptoms, including seizures, altered
mental status, disturbances in vision, ataxic gait, or motor and
sensory insufficiencies (17, 18). Vascular RCVS imaging may
indicate the alternating diffusing pattern of dilation and
constriction of cerebral artery vessels while excluding other
differentials (19). Additionally, 25–33% of RCVS patients may
experience various complications, such as PRES, cortical
subarachnoid hemorrhage, ischemic stroke, and intracranial
hemorrhage (5, 20).
FIGURE1
T2-FLAIR MRI revealed hyperintensities in the temporal, parietal, occipital lobes, left caudate nucleus, and right cerebellum (A). An MRA indicated
multifocal, segmental, and diuse narrowing of cerebral arteries (large and medium) [(B), red arrows]. After 10 days, complete remission of
abnormalities was observed in a follow-up MRI compared to the previous signals (C). An MRA demonstrated significant cerebral artery recovery from
diuse segmental constriction (D).
FIGURE2
Axial and coronal gadolinium-enhanced T1-weighted MR images
showed diuse pachymeningeal enhancement [(A,B), red arrows].

Li et al. 10.3389/fneur.2023.1281074
Frontiers in Neurology 04 frontiersin.org
Advancement in imaging methods and clinical decision-
making has made RCVS more recognizable. However, precisely
diagnosing this disease could be challenging. The differential
diagnosis of cerebral arteriopathies often includes RCVS and
primary angiitis of the CNS (PACNS). PACNS is treated with
immunosuppressive drugs for a prolonged time, but steroids in
RCVS could have deleterious effects (21). Female gender,
migraines, and postpartum are usual in RCVS patients.
Additionally, TCH, infarcts at the border regions, cortical SAH,
and vasogenic edema highly predict RCVS. However, PACNS
patients may have several tiny and deep infarcts, white matter
lesions that are quite deep, lesions resembling tumors, or various
lesions elevated by gadolinium (9, 22). Furthermore, an RCVS
2
score of ≥5 demonstrated high sensitivity and specificity for
detecting RCVS, while a ≤ 2 effectively excluded it (23). Vasogenic
edema resembling PRES can beidentified across 9–38% of RCVS
patients, consistent with our case (24). Over one-third of RCVS
cases possess increased headache-induced blood pressure.
Enhanced pressure of blood within the arteries and
vasoconstrictions in the major cerebral vessels were essential
PRES identifiers in such patients (25, 26).
No official guidelines exist for RCVS therapy. RCVS patients
must avoid headache triggers, withdraw precipitating vasoactive
agents, and require calcium channel antagonists to reduce TCH
(25). In our case, intracranial hypotension may bethe primum
movens of RCVS. Most patients with intracranial hypotension
respond to a short conservative management course followed by
non-targeted patching of epidural blood (27). RCVS drug
treatment with antagonists of calcium ion channels (nifedipine,
verapamil, or nimodipine) positively impacts the clinical
progression. However, glucocorticoids can lead to adverse
outcomes (24).
Conclusion
In summary, new-onset headaches should becarefully assessed
for secondary headaches during the peripartum period. is study
presented a postpartum RCVS patient aer intracranial hypotension
due to a CSF leakage caused by an inadvertent epidural puncture.
us, due to cerebral vasospasm, intracranial hypotension could
trigger RCVS.
Data availability statement
e raw data supporting the conclusions of this article will
bemade available by the authors, without undue reservation.
Ethics statement
e studies involving humans were approved by Institutional
Ethics Committee of Mianyang Central Hospital. e studies were
conducted in accordance with the local legislation and institutional
requirements. e patients/participants provided their written
informed consent to participate in this study. Written informed
consent was obtained from the participant/patient(s) for the
publication of this case report.
Author contributions
SL: Writing – original dra, Writing – review & editing, Data
curation, Soware. YY: Writing – original dra, Writing – review &
editing, Conceptualization. JZ: Investigation, Methodology, Soware,
Writing – review & editing. ND: Formal analysis, Project administration,
Writing – review & editing. GK: Conceptualization, Resources,
Supervision, Validation, Visualization, Writing – original dra.
Funding
e author(s) declare that no nancial support was received for
the research, authorship, and/or publication of this article.
Conflict of interest
e authors declare that the research was conducted in the
absence of any commercial or nancial relationships that could
beconstrued as a potential conict of interest.
Publisher’s note
All claims expressed in this article are solely those of the authors
and do not necessarily represent those of their aliated organizations,
or those of the publisher, the editors and the reviewers. Any product
that may be evaluated in this article, or claim that may be made by its
manufacturer, is not guaranteed or endorsed by the publisher.
FIGURE3
RCVS2 score for reversible cerebral vasoconstriction syndrome.

Li et al. 10.3389/fneur.2023.1281074
Frontiers in Neurology 05 frontiersin.org
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