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24 Headache Attributed toCerebral Venous Thrombosis (CVT)
233
24.5 Pain Pathways Involved inCVT-Related Headaches
The pain mechanisms underlying CVT-related headaches are multifaceted, involving both peripheral and central sensitization. The activation of the trigeminovascular system, combined with secondary inammatory and vascular changes, leads to
increased excitability of central pain-processing networks in the brainstem and
thalamus. This central sensitization contributes to headache chronicity and may
explain why some CVT patients develop prolonged post-thrombotic headaches
even after recanalization [16].
Given the complex interplay of venous congestion, neuroinammation, cerebral
edema, and hemorrhagic transformation, CVT-related headaches require a tailored
treatment approach [17]. Management strategies should focus on anticoagulation to
prevent thrombosis progression, symptomatic relief with analgesics, and measures
to reduce ICP, such as acetazolamide or therapeutic lumbar punctures (LPs) in
select cases. Early recognition and intervention are crucial to mitigate long-term
neurological complications and improve patient outcomes.
24.6 Clinical Characteristics
Cerebral venous thrombosis (CVT) is a complex condition that often presents with
headache as a primary symptom, occurring in up to 90% of cases [1]. The headache
associated with CVT can exhibit diverse characteristics, making its diagnosis challenging [18]. CVT-related headaches typically have a subacute or progressive onset,
though some cases may present with a thunderclap headache pattern, which is sudden and severe, reaching its peak within minutes [1, 19, 20]. Thunderclap headaches
in CVT can be particularly concerning, as they may mimic subarachnoid hemorrhage [7, 21]. The headache can be diffuse or localized, sometimes described as
throbbing or band-like [19, 22]. Diffuse headaches are common, affecting the whole
head, while localized headaches may involve specic regions such as the frontal,
temporal, or occipital areas [21]. CVT-related headaches are often accompanied by
nausea, photophobia, and focal neurological signs such as seizures, motor weakness, or visual disturbances [1]. These symptoms can vary widely among patients
and may not always be present.
Comparison with Other Secondary Headaches CVT-related headaches can be
confused with other secondary headache disorders due to overlapping symptoms.
For example, idiopathic intracranial hypertension (IIH) also presents with headache
and increased intracranial pressure, but typically includes visual disturbances like
papilledema [6]. Meningitis may also cause headache, but is usually accompanied
by fever and neck stiffness [23]. Stroke can present with a sudden-onset headache,
but is typically associated with focal neurological decits [23].
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D. Uluduz and A. Özge
Red ags for CVT include the sudden onset of severe headache, seizures, or
focal neurological decits such as weakness or visual disturbances [1, 2, 24]. These
symptoms necessitate prompt imaging and diagnosis to prevent complications and
improve outcomes.
24.7 Diagnosis ofCVT-Related Headache
Diagnosing cerebral venous thrombosis (CVT)-related headaches involves a combination of clinical suspicion and differential diagnosis, as the symptoms can mimic
other conditions such as idiopathic intracranial hypertension or subarachnoid hemorrhage [25, 26]. The headache associated with CVT often presents with nonspecic features, making it crucial to consider CVT in the differential diagnosis of
patients with headache, especially when accompanied by signs of increased intracranial pressure or focal neurological decits [25].
Clinical Presentation and Pathophysiology The headache associated with CVT is
highly variable and can range from a diffuse, pressure-like pain to a thunderclap
headache suggestive of subarachnoid hemorrhage. The underlying mechanism is
related to impaired venous drainage, leading to increased intracranial pressure
(ICP), cerebral congestion, and potential venous infarction [26]. The increased ICP
results from obstructed cerebrospinal uid (CSF) absorption at the arachnoid granulations due to venous hypertension, contributing to persistent headaches, often exacerbated by Valsalva maneuvers, coughing or postural changes.
Focal neurological decits such as hemiparesis, aphasia, or seizures may arise
from localized venous infarcts due to thrombosis-induced ischemia. The presence
of papilledema on fundoscopic examination can further support the diagnosis of
raised ICP secondary to CVT.In some cases, patients may also present with altered
mental status or encephalopathy, particularly if there is signicant cerebral edema
or hemorrhagic transformation [25].
Neuroimaging Approaches
Neuroimaging plays a pivotal role in the diagnosis of
CVT.The gold standard imaging modality is magnetic resonance imaging (MRI)
combined with magnetic resonance venography (MRV), which provides high sensitivity and specicity in detecting thrombosed cerebral veins or dural sinuses [1].
MRI sequences such as susceptibility-weighted imaging (SWI) and T2* gradient
echo can help visualize the thrombus by detecting deoxygenated blood products. In
contrast, contrast-enhanced MRV can delineate the extent of venous obstruction.
Computed tomography (CT) and CT venography (CTV) can serve as alternatives,
particularly in emergency settings where MRI may not be immediately available. A
hyperdense appearance of the thrombosed sinus on non-contrast CT (commonly
observed in the superior sagittal sinus) or the “empty delta sign” on contrastenhanced CT suggests CVT [26]. However, CT imaging alone may miss early or
partial thrombosis, necessitating further conrmation with MRV. In ambiguous
24 Headache Attributed toCerebral Venous Thrombosis (CVT)
235
cases, digital subtraction angiography (DSA) may be considered as a conrmatory
tool to visualize venous outow patterns and detect subtle occlusions not delineated
by MRV or CTV [27].
Lumbar Puncture and CSF Analysis in CVT While lumbar puncture (LP) is not a
primary diagnostic tool for CVT, it can provide supportive evidence by revealing
elevated opening pressure, which indicates increased ICP [25]. Elevated protein
levels and mild pleocytosis in the CSF may suggest secondary inammatory or
ischemic processes. However, normal CSF ndings do not exclude CVT, and LP
should be performed cautiously in the presence of signicant intracranial hypertension to prevent brain herniation.
Biomarkers and Laboratory Tests for Prothrombotic States Since CVT often
occurs in the setting of an underlying hypercoagulable state, laboratory investigations are essential to identify potential risk factors. Prothrombotic screening should
include testing for inherited and acquired thrombophilic disorders such as: Factor V
Leiden mutation (leading to activated protein C resistance), prothrombin gene
mutation (G20210A), antithrombin III, protein C, and protein S deciencies
(impairing anticoagulant pathways), antiphospholipid syndrome (testing for lupus
anticoagulant, anticardiolipin, and β2-glycoprotein-I antibodies), elevated homocysteine levels, which can predispose to endothelial dysfunction and thrombosis
[26]. Additional inammatory markers, such as D-dimer, can assist in early screening, as elevated levels indicate ongoing brinolysis in the presence of a thrombus.
However, D-dimer levels may be normal in isolated cortical vein thrombosis or
subacute CVT, necessitating further imaging conrmation [26].
24.8 Treatment Approaches
The cornerstone of CVT treatment is anticoagulation therapy, which aims to prevent
clot propagation, facilitate recanalization, and reduce venous congestion.
Unfractionated heparin (UFH) is used in the acute phase, particularly in critically ill
patients, as it offers the advantage of rapid reversal in cases of hemorrhagic complications. UFH is administered intravenously and requires dose adjustments based on
activated partial thromboplastin time (aPTT) values [27, 28]. Low-molecular weight
heparin (LMWH) is preferred over UFH due to its more predictable pharmacokinetics, lower risk of heparin-induced thrombocytopenia, and reduced need for monitoring. LMWH has been shown to have better efcacy and safety compared to UFH in
CVT treatment [9]. Warfarin is initiated after the acute phase, usually as a bridging
therapy following heparin, targeting an international normalized ratio (INR) of 2–3.
Warfarin has traditionally been used for long-term anticoagulation in CVT [9].
Direct oral anticoagulants (DOACs) are increasingly considered as alternatives to
warfarin due to their ease of use, xed dosing, and lower bleeding risk. Recent studies suggest comparable efcacy of DOACs in CVT treatment, but more extensive
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D. Uluduz and A. Özge
trials are needed for denitive guidelines [29]. Notably, anticoagulation is recommended even in the presence of intracranial hemorrhage, as CVT-induced bleeding
often results from venous congestion rather than primary vessel rupture. The rationale for continued anticoagulation is to reduce venous hypertension, thereby preventing further hemorrhage and facilitating clot resolution.
In cases where anticoagulation therapy fails or neurological deterioration progresses despite optimal medical management, endovascular treatment may be considered. These interventions include mechanical thrombectomy, like catheter-based
techniques that physically remove thrombi from the occluded venous sinuses,
restoring venous drainage and reducing ICP [30]. Catheter-directed thrombolysis
involves direct administration of thrombolytic agents (e.g., recombinant tissue plasminogen activator, rtPA) into the thrombus to accelerate clot dissolution [31].
Angioplasty with stenting is rarely performed, but is considered in cases with persistent venous sinus stenosis contributing to ongoing venous hypertension [26, 32,
33]. Endovascular interventions remain reserved for severe cases where conven-
tional anticoagulation fails, particularly in the setting of extensive cerebral edema or
coma. In severe cases with signicant neurological deterioration, endovascular
interventions such as mechanical thrombectomy or thrombolysis may be considered
to restore venous outow and alleviate intracranial hypertension [26, 32].
Management of Increased Intracranial Pressure (ICP) Elevated ICP is a major
contributor to CVT-related headaches and may lead to secondary neurological
deterioration if not effectively controlled. Acetazolamide, a carbonic anhydrase
inhibitor, reduces cerebrospinal uid (CSF) production, thereby lowering ICP.It is
commonly used in patients with signs of intracranial hypertension, such as papilledema. Acetazolamide has been shown to have an acceptable safety prole at dosages up to 4 g/d in treating idiopathic intracranial hypertension, though it is
associated with side effects like paresthesia and dysgeusia [34, 35]. Agents such as
mannitol and hypertonic saline can be administered to reduce cerebral edema in
acute settings. Both agents are effective in reducing ICP, but hypertonic saline may
offer a greater and longer-lasting effect compared to mannitol. Corticosteroids are
not routinely recommended unless there is an associated inammatory component,
such as vasculitis or autoimmune thrombosis. Corticosteroids have shown minimal
benet in reducing ICP in conditions like traumatic brain injury and ischemic stroke.
Still, they may be benecial in bacterial meningitis by reducing complications like
sensorineural hearing loss [36]. In cases of refractory increased ICP with impending
brain herniation, decompressive craniectomy or ventriculostomy may be required to
relieve pressure and prevent fatal outcomes. Decompressive craniectomy has been
shown to improve survival and functional outcomes in patients with malignant cerebral infarctions by effectively controlling ICP [37].
Analgesics and anti-inammatory drugs are used for the symptomatic relief of
headaches. However, prolonged use of nonsteroidal anti-inammatory drugs
(NSAIDs) should be avoided due to bleeding risks when combined with anticoagulation [10–13]. The use of corticosteroids is controversial and generally not recommended unless there are specic indications, such as signicant inammation. In
24 Headache Attributed toCerebral Venous Thrombosis (CVT)
some cases, CVT-related headaches may mimic migraines, and similar treatment
strategies may be employed.
Some patients may experience persistent or recurrent headaches post-CVT,
which can evolve into chronic daily headache or post-thrombotic headache syndromes [13]. The risk of developing chronic headache conditions is a concern,
necessitating long-term follow-up and management [13]. CVT can lead to cognitive
impairments and neuropsychological issues, which require rehabilitation efforts to
improve quality of life [8]. Patients should be advised on lifestyle modications to
prevent future episodes, including management of prothrombotic conditions and
avoiding triggers for headache exacerbation.
237
24.9 Future Directions andResearch Gaps
Despite signicant advances in the understanding and management of cerebral
venous sinus thrombosis (CVT) and its associated headaches, several gaps remain
that warrant further research and clinical innovation. The identication of reliable
biomarkers could facilitate earlier diagnosis of CVT, reducing delays in treatment
initiation. Research into inammatory markers, coagulation proles, and endothelial dysfunction could provide valuable insights into CVT pathophysiology and aid
in risk stratication. CVT-related headaches exhibit substantial heterogeneity, ranging from migraine-like to tension-type or thunderclap headaches. Tailoring treatment strategies based on individual headache phenotypes could enhance symptom
control and improve patient quality of life. While acute treatment is well- established,
there is a need for structured long-term follow-up protocols to monitor recurrence
risk, persistent headaches, and cognitive or psychological sequelae in CVT survivors. Future studies should explore optimal surveillance intervals and preventive
strategies.
24.10 Conclusion
Cerebral venous sinus thrombosis remains a complex and potentially life- threatening
condition, often presenting with persistent and debilitating headaches. Early recognition and accurate diagnosis are crucial to initiating timely anticoagulation therapy
and managing intracranial hypertension effectively.
This review highlights the importance of neuroimaging as the cornerstone of
CVT diagnosis, the role of anticoagulation in preventing disease progression, and
the need for personalized approaches in headache management. The long-term burden of CVT-related headaches underscores the necessity for continued research into
biomarkers, neuroimaging techniques, and individualized treatment protocols.
Given the multifaceted nature of CVT, an interdisciplinary approach involving neurologists, hematologists, radiologists, and other specialists is essential to optimize
238
D. Uluduz and A. Özge
patient outcomes. Future studies should focus on rening diagnostic tools, improving therapeutic strategies, and developing comprehensive long-term care models to
enhance the quality of life for CVT survivors.
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D. Uluduz and A. Özge
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Chapter 25
Headache Attributed toReversible
Cerebral Vasoconstriction Syndrome
MarceloValença andLucianaPatríziaAlvesde Andrade-Valença
25.1 Introduction
Reversible cerebral vasoconstriction syndrome (RCVS) is a condition characterized
by recurrent episodes of thunderclap headache associated with reversible multifocal
vasoconstriction of cerebral arteries [1–3]. The syndrome can occur spontaneously
or be triggered by various factors, including sexual activity, antidepressants such as
selective serotonin reuptake inhibitors [4, 5], immunosuppressant pharmacotherapy
[6–8], vasoactive drugs [9], cocaine abuse [10], bromuscular dysplasia [11],
snakebite victims [12], Guillain–Barré syndrome [13], coronavirus 2 vaccination
[14], intracranial hypotension [15], among other causes [16]. The female sex
appears to be more susceptible to RCVS [17]. Cases of RCVS have been diagnosed
as a rare and understudied transfusion reaction, most commonly observed in adult
females after the correction of chronic, severe anemia [18, 19]. While initially
underrecognized, RCVS has gained increasing attention due to its potential to cause
severe neurological complications.
The rst time we encountered a patient with RCVS was in 1999. She was a
woman who experienced two episodes of explosive thunderclap headache occurring
during separate orgasms, one during sexual intercourse and another during masturbation. Her case was published in 2004 alongside a literature review of similar cases
associated with sexual activity [20]. In our article, we mentioned that we found
several cases in the literature of thunderclap headache with concurrent intracranial
arterial vasoconstriction unrelated to eclampsia, suggesting a distinct clinical entity.
We rst introduced this concept at the Brazilian Headache Congress in 2002 [21],
where we proposed it as a new headache syndrome, initially named “Abrupt Severe
Headache Associated with Segmental Artery Narrowing (ASHCAN).” At that time,
we drafted an article outlining diagnostic criteria, clinical features, and prognostic
M. Valença (*) · L. P. A. de Andrade-Valença
Federal University of Pernambuco, Recife, Brazil
Switzerland AG 2026
D. Uludüz et al. (eds.), Rare Causes of Headache Disorders, Headache,
https://doi.org/10.1007/978-3-032-10242-3_25
241© The Author(s), under exclusive license to Springer Nature
242
M. Valença and L. P. A. de Andrade-Valença
factors. However, the article was not accepted for publication, with no justication
provided. Years later, we eventually published our ndings, contributing to the
growing recognition of RCVS as a distinct neurological disorder [16].
25.2 Case Presentation
A 42-year-old woman with a history of chronic migraine-like headache and overuse
of analgesics, particularly Neosaldina® (a combination of isometheptene, dipyrone
sodium, and caffeine), was scheduled for elective gallbladder surgery. One week
before the procedure, she took Allegra D (fexofenadine) for 7days to manage her
allergy symptoms.
During the surgery, she developed anaphylactic shock, requiring resuscitation.
Following this event, she became torpid and exhibited right-sided hemiparesis.
Magnetic resonance imaging (MRI) with angiography revealed multiple ischemic
lesions affecting different arterial territories (Fig.25.1), and MR angiography showed
diffuse arterial narrowing in several cerebral arteries (Fig.25.2), consistent with RCVS.
The patient deteriorated further, developing intracranial hypertension, primarily
due to cerebellar edema with brainstem compression. MRI scans revealed the
absence of subarachnoid cisterns, indicating a signicant mass effect in the posterior fossa (Fig.25.1b, c). An emergency posterior fossa decompressive craniectomy
with duroplasty was performed to relieve pressure (Fig.25.3).
Over the next 2months, the patient showed gradual improvement in consciousness,
initially presenting with akinetic mutism, where she failed to open her eyes but occasionally obeyed simple verbal commands. At 6months, she regained the ability to
walk and communicate verbally, though she remained somewhat apathetic with mild
right-arm motor weakness. She was even able to drive under her husband’s supervision.
abc
Fig. 25.1 Axial brain MRI showing ischemic areas in the left posterior cerebral artery territory,
including the left thalamus (a), and a large cerebellar ischemic area involving both hemispheres
with signicant brainstem compression (b and c). Note the near-complete disappearance of the
posterior fossa cisterns, suggesting severe compartmental hypertension